Paediatrics Flashcards
1
Q
Name some common viral causes of RESPIRATORY infections (80-90% of infections are viral)
A
- Respiratory syncytial virus (RSV)
- Rhinovirus
- Influenza
- Metapneumovirus
- Adenoviruses
2
Q
Name some common bacterial causes of RESPIRATORY infections
A
- Strep penumoniae
- haemophilus influenza
- Moraxella catarrhalis
- Bordetella pertussis
- Mycoplasma pneumoniae
3
Q
Risk factors of RESPIRATORY infections?
A
- Parental smoking
- Poor socioeconomic status (damp/ overcrowded)
- Poor nutrition
- Male gender
- Immunodeficiency
Underlying condition
4
Q
Give some examples of URTIs and what they can cause
A
- Coryza (cold)
- Sore throat (pharyngitis, tonsillitis)
- Acute otitis media
- Sinusitis
Cause:
Difficulty feeding
Febrile convulsions
- Asthma exacerbations
5
Q
Describe symptoms of the common cold
Common pathogens
and the treatment
A
- Clear/ mucopurulent nasal discharge and nasal blockage
Common pathogens:
Rhinovirus
coronaviruses
RSV
Paracetamol
Ibruprofen
6
Q
Common pathogens and symptoms of Pharyngitis
A
Viral
- Common cold viruses
- Adenoviruses
- EBV
Bacterial (older children)
- Group A beta-haemolytic strep
Pharynx and soft palate inflamed
Local lymph nodes enlarged and tender
7
Q
What is Tonsillitis?
A
Form of pharyngitis where there is intense inflammation of the tonsils often with purulent exudate
8
Q
What is the CENTOR criteria?
A
- Tonsillar exudate
- Tender anterior cervical lymphadenopathy
- Fever
- Absence of cough
3+ of these criteria = Strep infection- Abx
9
Q
What is Quinsy?
A
Peritonsillar abscess (complication of tonsillitis)
10
Q
Give some symptoms of Quinsy
A
- Sore throat, dysphagia, uvula deviation, trismus (lockjaw)
11
Q
Management of tonsillitis?
A
Symptomatic relief : Para and Ibruprofen
Penicillin V or Erythromycin
Tonsillectomy in recurring cases
12
Q
What is Acute Otitis media and why is it common in children?
A
Infection of the middle ear due to short, horizontal eustachian tubes and mucal discharge almost always the middle ear
13
Q
Presenting complaint of Acute Otitis media?
A
- Rapid onset ear pain (due to bulging of tympanic membrane)
Pyrexia/fever
Otorrhoea
14
Q
Management of Otitis media?
A
> 4 days of no symptoms: Amoxicillin / Erythromycin
Grommet insertion if recurrent
15
Q
What is the leading cause of hearing loss in children?
A
Otitis media with effusion or glue ear
16
Q
management of sinusitis?
A
Amoxicillin/ doxycycline
Avoid antihistamines as it may thicken secretions
17
Q
Signs of upper airway obstruction?
A
- Stridor
- Hoarseness
- Barking cough (sea lion)
- Dyspnoea
18
Q
How is the severity of upper airway obstruction assessed?
A
Degree of chest retraction and degree of stridor
19
Q
What should you avoid in a suspected upper airway obstruction?
A
Avoid examination using a spatula as this may precipitate total obstruction
20
Q
Describe why hoarseness occurs
A
Inflammation of the vocal cords
21
Q
What is stridor?
A
Rasping sound heard predominantly on inspiration
22
Q
CROUP
What is it otherwise known as?
A
Laryngotracheobronchitis
23
Q
CROUP
What is it?
A
Mucosal inflammation and increased secretions that affect the airway
24
Q
CROUP
Where is oedema dangerous?
A
Subglottic area as this may result in critical narrowing of the trachea
25
CROUP
Viral causes?
Parainfluenza 1, 2, 3 (most common)
Metapneumovirus
RSV
Influenza
26
CROUP
When is it most common?
Autumn time, between the ages of 6 months to 6 years with a peak incidence around 2 years old
27
CROUP
Clinical presentation?
- Barking cough (worse at night)
- Harsh stridor
- Hoarseness
- Preceding non specific viral URTI
(coryza, fever, cough)
WORSE AT NIGHT
28
CROUP
Signs of severe disease?
Cyanosis
Altered consciousness
Rising HR/RR
Restlessness
29
What is Bacterial Tracheitis?
Pseudomembranous croup is an uncommon but dangerous disease very similar to severe croup but with:
- High fever
- Appears toxic
- Tracheal tenderness
- Rapidly progressive airways obstruction (thick exudate cant be cleared by coughing)
30
What is bacterial tracheitis caused by and what is the management?
S.aureus, Strep A, haemophilus
IV Abx
31
What is Acute Epiglottitis and what is it caused by?
Life threatening emergency due to high risk of respiratory obstruction caused by:
Haemophilus influenza type B
32
What affect has the Hib immunisation had an the incidence of epiglottitis?
>99% reduction in cases, most common in ages 2-7 years
33
Clinical presentation of Acute Epiglottitis?
- Intense swelling of epiglottis
- Onset very acute
- High fever
- Dysphagia and speech difficulty due to pain
- Drooling
- Stridor
- Minimal cough
34
In what position would you expect a child with trachea obstruction to be in?
Immobile, upright, with an open mouth to optimise the airway
35
What should you never do when suspecting obstruction of the respiratory tract/ acute epiglottitis?
Do not examine throat with spatula or lie the child down
Do not upset or cannulate
36
Management of Acute Epiglottitis?
IV Abx- Cefotaxime
Intubate
Tracheostomy may be required if complete obstruction
37
What is the difference between Croup and Acute Epiglottitis in terms of:
1) Onset
2) Cough
3) Preceding coryza
4) Able to drink
Croup
1) onset: days
2) Severe barking cough
3) preceding coryza
4) can drink
Acute Epiglottitis
1) onset: hours
2) absent/ minor cough
3) no preceding coryza
4) cant drink
38
What is the difference between Croup and Acute Epiglottitis in terms of:
1) Drooling?
2) Appearance of child
3) Fever
4) Stridor and voice/cry?
Croup
1) no drooling
2) unwell appearance
3) fever <38.5
4) harsh stridor with hoarse cry
Acute Epiglottitis
1) drooling
2) toxic, very unwell
3) fever >38.5
4) soft whsipering stridor, muffled cry/ reluctant to speak
39
WHOOPING COUGH (PERTUSSIS)
What is it?
NOTIFIABLE DISEASE
Acute, highly contagious resp infection transmitted by respiratory droplets
Co- infection with RSV (Bronchiolitis) common
40
WHOOPING COUGH (PERTUSSIS)
Causing agent?
Bordetella pertussis, gram negative coccobacillus
41
WHOOPING COUGH (PERTUSSIS)
How long do symptoms last and what is the first stage?
6-8 weeks and the first stage is the
Catarrhal Stage
42
WHOOPING COUGH (PERTUSSIS)
Clinical presentation of the Catarrhal stage?
(1st stage)
- Malaise
- Conjunctivitis
- Nasal discharge
- Sore throat
- Dry cough
- Mild fever
43
WHOOPING COUGH (PERTUSSIS)
What is the second stage?
After two weeks of the catarrhal phase it becomes the
Paroxysmal coughing stage
44
WHOOPING COUGH (PERTUSSIS)
Describe the Paroxysmal coughing stage
Dry hacking cough that is worse at night and after feeds
coughing episodes folllowed by characteristic 'whoop'
45
WHOOPING COUGH (PERTUSSIS)
What is the characteristic 'whoop'?
Inspiration against a closed glottis,
Child chokes, gasps and face reddens
46
WHOOPING COUGH (PERTUSSIS)
What can happen post cough?
Vomiting,
Apnoea
Cyanosis
subconjunctival haemorrhage/ anoxia can be brought on by coughing fits, leading to seizures/syncope
47
WHOOPING COUGH (PERTUSSIS)
Diagnostic tests?
- Notifiable disease
- PCR via nasal swab
- Nasopharyngeal swabs
- test for anti- pertussis IgG
- Marked lymphacytosis= COMMON
48
WHOOPING COUGH (PERTUSSIS)
treatment?
- Hospitalised 1%- if <6 months (risk of apnoea)
10-14 days incubation
Macrolides 1st Line:
Azithromycin or Clarithromycin or
Erythromycin
Erythromycin for pregnant women
Off school for 48 hours after Abx commencement
49
BRONCHIOLITIS
What is it?
commonest lung infection in infants
rare after 1 years of age and peak 3-6 months
50
BRONCHIOLITIS
what makes this more severe?
Congenital heart defects
Cystic Fibrosis
Down's
51
BRONCHIOLITIS
Cause?
RSV in 80%of cases
( Respiratory syncytial virus)
Metapneumovirus
Parainfluenza
Rhinovirus
Adenovirus
52
BRONCHIOLITIS
Cause of severe bronchiolitis?
RSV and Metapneumovirus dual infection is associated with severe bronchiolitis
53
BRONCHIOLITIS
Risk factors?
- Premature/ low birth weight
- Cystic Fibrosis
- Congenital heart disease
- Down's
- Immunocompromised
54
BRONCHIOLITIS
Clinical presentation?
- Coryza precedes dry cough
- Increasing breathlessness
- feeding difficulties
- fever
- tachycardia
55
BRONCHIOLITIS
When should a child be admitted?
- Infrequent feeding
- Respiratory distress
- hypoxia
56
BRONCHIOLITIS
Diagnostic tests and results?
- Pulse oximetry
- viral throat swab
Not routine:
CXR- exclude pneumothorax
blood gas analysis, FBC
57
BRONCHIOLITIS
Treatment?
Oxygen therapy
Supportive (NG nutrition, fluids, temp control)
ventilation (CPAP)
58
BRONCHIOLITIS
What is Ribavirin?
Antiviral meds against RSV,
Used for Hep C with (interferons/ simeprevir, sofosbuvir)
and some haemorrhagic fevers
59
BRONCHIOLITIS
What is Ribavirin?
Antiviral meds against RSV,
Used for Hep C with (interferons/ simeprevir, sofosbuvir)
and some haemorrhagic fevers
60
BRONCHIOLITIS
Signs on examination
wheeze and end inspiratory crackles
Cyanosis
Sub/ intercostal recession
Hyperinflation of chest
61
BRONCHIOLITIS
Associated signs with hyperinflation of the chest
Prominent sternum
Liver displaced downwards
62
What should be given if SpO2 <92%?
High flow humidified oxygen via nasal cannulae
63
PNEUMONIA
Cause in:
Newborns?
Group B strep- maternal
Gram negative enterococci
64
PNEUMONIA
Cause in:
Infants and young children?
Strep pneumoniae
haemophilus influenza B
Staph A (infrequent but serious)
Mycobacterium tuberculosis
65
PNEUMONIA
Cause in:
Viral cases?
RSV
Influenza A/B
Parainfluenza
Adenovirus
66
PNEUMONIA
Cause in:
Atypical?
Mycoplasma pneumoniae
Chlamydia pneumoniae
67
PNEUMONIA
Diagnostic tests?
- Cough
- fever
- lethargy
- poor feeding
- respiratory distress
68
PNEUMONIA
Symptoms of respiratory distress?
```
tachypnoea
cyanosis
grunting
intercostal recession
use of accesory muscles
nasal flaring
```
69
PNEUMONIA
Tests for severe?
blood cultures
sputum culture
FBC, CRP
CXR
70
PNEUMONIA
Signs in older children?
end inspiratory crackles
bronchial breathing
pleuritic pain
71
PNEUMONIA
Treatment?
If resp distress/ O2 <92%- admit to hospital
- Oxygen
- IV fluids
- IV Abx for newborns
72
PNEUMONIA
What Abx are 1st line?
Amoxicillin
Alternatives:
Clarithromycin
Co-amoxiclav
Azithromycin
73
ASTHMA
What are the two types of wheeze?
transient early wheeze
persistent and recurrent wheezing
74
ASTHMA
What is transient early wheeze?
viral associated wheeze- RSV
small airways obstructed due to inflammation
more common in males and resolves by 5 years of age
75
ASTHMA
What is persistent and recurrent wheeze?
Presence of IgE to common inhalant allergens (Atopic Asthma)
Common in family Hx and associated with eczema, hayfever, food allergies
76
ASTHMA
Pathophysiology?
Bronchial inflammation leads to:
1) Oedema
2) Excessive mucous production
3) Infiltration of cells
77
ASTHMA
What does bronchial inflammation lead to?
Airway narrowing and reversible airflow obstruction
78
ASTHMA
What cells infiltrate the bronchials
Eosinophils
Neutrophils
Mast cells
Lymphocytes
79
ASTHMA
Risk factors?
```
Low BW
Family Hx
Bottle fed
Atopy
Male
Pollution
```
ADAM33 Gene
80
ASTHMA
Triggers?
```
Pollen
Dust
Feathers
Exercise
Pollution
Cold air
Illness (viruses)
```
81
ASTHMA
Diagnostic tests?
CXR to rule out other causes
Spirometry
Peak flow shows diurnal variation
82
ASTHMA
Clinical presentation?
Polyphonic wheeze (expiratory- obstruction)
Coughing
SOB
Symptoms worse at night and morning
SILENT CHEST RED FLAG- exacerbation
83
ASTHMA
treatment for ages 5-16
Step 1: SABA (salbutamol)
Step 2: SABA + low dose ICS (beclomethasone)
Step 3: SABA+ ICS + LTRA (Montelukast)
Step 4: SABA+ ICS + LABA (salmeterol)
Step 5:
SABA + low dose MART
(ICS and fast acing LABA- formoterol)
Step 6: SABA + moderate dose MART
84
ASTHMA
Treatment to under 5
1) SABA
2) 8 week moderate dose ICS trial
3) Add LTRA
85
ASTHMA
Treatment to severe asthma attack?
O SHIT ME
Oxygen
Salbutamol (nebulised)
Hydrocortisone IV/ oral prednisolone
Ipratropium bromide (nebulised)
Theophylline IV
Magnesium sulphate IV
Escalate
86
What is Kartagener syndrome?
rare, autosomal recessive genetic ciliary disorder comprising the triad of situs inversus, chronic sinusitis, and bronchiectasis.
This leads to recurrent chest infections
87
CYSTIC FIBROSIS
What is it?
Alteration of the viscosity and tenacity of mucous produced at epithelial surfaces
88
CYSTIC FIBROSIS
What is the classic form of CF?
Bronchiopulmonary infection and pancreatic insuffieciency with a high sweat sodium and chloride concentration
89
CYSTIC FIBROSIS
Defective gene?
Mutations with cystic fibrosis transmembrane conductance regulator gene on chromosome 7
(CFTR)
90
CYSTIC FIBROSIS
How is it caused?
CFTR gene is for a critical chloride channel,
1) Decreased excretion of chloride into the airway lumen and an increased reabsorption of sodium into the epithelial cells
2) With less excretion of salt there is less excretion of water and increased viscosity/ tenacity of airway secretion
91
CYSTIC FIBROSIS
Cause?
Autosomal recessive
Commonest is a deletion at position 508 in amino acid sequence which results in a defect to the CFTR gene
92
CYSTIC FIBROSIS
Respiratory Clinical presentation?
Recurrent infections
Sinusitis
Nasal polyps
SOB and haemoptysis
Airflow limitation and
bronchiectasis
Respiratory failure
cor pulmonale (RVH - late sign)
93
CYSTIC FIBROSIS
GI Clinical presentation?
Steatorrhoea due to pancreatic dysfunction
10% meconium ileus
cholesterol gallstones
Increased incidence of peptic ulceration and GI malignancy
94
CYSTIC FIBROSIS
Nutritional effects?
- Malnutrition due to malabsorption
| - Failure to thrive and slow growth
95
CYSTIC FIBROSIS
Other Clinical signs?
signs in a male/female?
Puberty and skeletal maturity delay
- Male almost always infertile
- Secondary amenorrhoea in females
Arthropathy and DM in 11% of adults with CF
96
CYSTIC FIBROSIS
Diagnostic tests?
All UK newborns screened
SWEAT TEST:
- sodium conc >60mmol/L
97
CYSTIC FIBROSIS
Antibiotic Treatment?
younger: s.aureus, strep. pneumoniae
EVENTUALLY >90%:
Pseudomonas aeruginosa,
(nebulised anti-pseudomonal Ab therapy helps improve lung function)
98
CYSTIC FIBROSIS
Drug therapy?
B2- agonists
ICS
Symptomatic relief
Lung transplant if FEV1 <30% expected
99
CYSTIC FIBROSIS
method of airway clearance?
inhalation of recombinant human deoxyribonuclease (rhDNAse)
hypertonic saline by inhalation
Amiloride (inhibits sodium transport)
Acetylcysteine
100
CYSTIC FIBROSIS
Pancreatic insufficiency treatment?
PERT
| pancreatic enzyme replacement therapy
101
CYSTIC FIBROSIS
Complications?
- Cirrhosis
- DM
- Haemoptysis
- Male infertility
- Pneumonia
- Pneumothorax
- Pulmonary osteoarthropathy
102
What are the 3 categories of Congenital Heart Disease?
1) Holes / connections
2) Narrowing / stenosis
3) Complex (right to left shunt)
103
Give some examples of 'holes/ connections of CHD?
ASD
PDA
VSD
AVSD
104
Give some examples of 'narrowing/ stenosis' of CHD?
Coarctation of the aorta (collapse with shock)
AS
PS
105
Give some examples of complex CHDs?
Tetralogy of Fallot
Transposition of the great arteries
106
What does right to left shunts cause?
Cyanosis
107
Signs of a possible CHD?
- Poor feeding
- Dyspnoea
- Tachycardia
- Cool peripheries
- Acidosis on ABG
108
Investigations of CHD
FBC. CXR, PaO2, Echo +/- cardiac catheterisation
109
ATRIAL SEPTAL DEFECT
Causes?
Foetal alcohol syndrome,
common in trisomy 21
110
ATRIAL SEPTAL DEFECT
Types?
- Ostium Primum 10%
- Ostium Secondum 90%
(10-15% of all CHDs)
- AVSD
111
Listening to S2:
If it is split (double) during inspiration and single during expiration what does it mean?
Normal!
112
Listening to S2:
If it is split (double) all the time what does it mean?
ASD
113
Listening to S2:
If it is never split (double) what does it mean?
All other CHDs
114
ATRIAL SEPTAL DEFECT
Clinical features?
Fixed and widely split S2
ejection systolic murmur in pulmonary area
115
ATRIAL SEPTAL DEFECT
Investigations and management?
CXR
ECG
Surgical correction
116
PATENT DUCTUS ARTERIOSIS
What is it?
tube between the aorta and pulmonary artery that fails to close after birth
117
PATENT DUCTUS ARTERIOSIS
Clinical features?
- poor feeding
- Tachypnoea
- Hepatomegaly
- Oedema
- Pneumonias
118
PATENT DUCTUS ARTERIOSIS
Severe clinical features?
CCF + pulmonary hypertension
119
PATENT DUCTUS ARTERIOSIS
Features on examination?
Large, bounding collapsing pulse
Heaving apex beat
Left subclavicular thrill
LOUD S2
gallop rhythm
120
PATENT DUCTUS ARTERIOSIS
murmur heard?
Classical continuous machinery murmur pulmonary area
121
PATENT DUCTUS ARTERIOSIS
Investigations?
ECG and CXR often normal
Echo- ensure no duct dependant circulation (e.g in pulmonary atresia)
122
PATENT DUCTUS ARTERIOSIS
management?
Ibuprofen/ Indomethacin (prostaglandin inhibitor) to close
Abolish lifelong bacterial endocarditis risk by SURGERY
123
VENTRICULAR SEPTAL DEFECT
what is there risk of?
HIGH risk of bacterial endocarditis
124
VENTRICULAR SEPTAL DEFECT
Clinical features?
Usually mild
- poor feeding
- Tachypnoea
- Hepatomegaly
- Oedema
125
VENTRICULAR SEPTAL DEFECT
Examination findings?
Thrill
Gallop rhythm
Active pre-cordium
126
VENTRICULAR SEPTAL DEFECT
What murmur is heard?
Harsh loud pansystolic mumur best heard in LLSE +/- thrill
127
VENTRICULAR SEPTAL DEFECT
Investigations?
Echo - precise defect
ECG - hypertrophy
CXR- cardiomegaly and enlarged PA + pulmonary oedema
128
VENTRICULAR SEPTAL DEFECT
Management?
Muscular VSDs 30% close spontaneously
Heart failure: Diuretics
additional calorie input
SURGERY to prevent damage to pulmonary capillary bed due to pulmonary HTN and high blood flow
129
ATRIOVENTRICULAR SEPTAL DEFECT
What is it common in?
Trisomy 21
130
ATRIOVENTRICULAR SEPTAL DEFECT
Clinical features?
- poor feeding
- Tachypnoea
- Hepatomegaly
- Oedema
- Failure to thrive
EXAM
- Thrill
- Gallop rhythm
131
ATRIOVENTRICULAR SEPTAL DEFECT
Examination findings?
Present at antenatal US scan
Cyanosis at birth/ heart failure at 2-3 weeks
No murmur
Often detected by echo check up in Down's
132
ATRIOVENTRICULAR SEPTAL DEFECT
Management?
Treat heart failure
Surgical repair at 3-6 months
133
COARCTATION OF THE AORTA
What is it?
Arterial duct tissue encirculing the aorta at the point it inserts into the duct:
1) Constricts aorta when duct closes
2) Obstruction to LV outflow
134
COARCTATION OF THE AORTA
Clinical features?
When ductus arteriosus closes; acute circulatory collapse
Weak femoral pulses
Radio-femoral delay
Heart failure
Metabolic acidosis
135
COARCTATION OF THE AORTA
Investigations and examination findings?
4 limb BP
Ejection systolic murmur
CXR- cardiomegaly
136
COARCTATION OF THE AORTA
Management?
Surgical repair by 5 years old
137
AORTIC STENOSIS
What is it?
Aortic valve leaflets partly fused together- restricted exit from LV
138
AORTIC STENOSIS
Clinical features?
- Ejection systolic murmur - URSE!
- Carotid thrill in suprasternal region
- Critical AS- severe heart failure
139
AORTIC STENOSIS
Investigations, findings and management?
Radiograph- prominent LV
ECG
- Regular echos
- balloon valvotomy/ dilation
Valve replacement
140
PULMONARY STENOSIS
What is it?
pulmonary valve leaflets partly fused together which restricts exit from the RV
141
PULMONARY STENOSIS
Clinical features?
Systolic ejection murmur in ULSE
right ventricular heave
142
PULMONARY STENOSIS
Investigations and management?
- ECG- upright T wave/ RV hypertrophy
CXR
Transcathether balloon dilation
143
TRANSPOSITION OF THE GREAT ARTERIES
What is the underpinning basic science?
1) Aorta connected to RV, pulmonary artery connected to LV (deoxygenated blood to body)
2) Incompatible with life (often have naturally occurring ASD,VSD, PDA)
144
TRANSPOSITION OF THE GREAT ARTERIES
Clinical features?
- Cyanosis
- S2 usually loud and singular
- Reduced sats
145
TRANSPOSITION OF THE GREAT ARTERIES
Investigation?
Check for 22q deletion
CXR- narrow upper mediastinum 'egg on side' appearance of cardiac shadow
Echo- abnormal arterial connections
146
TRANSPOSITION OF THE GREAT ARTERIES
Management?
Maintaining patency of ductus arteriosus is key- prostaglandin infusion
Balloon atrial septostomy
Surgery- arterial switch procedure in first few days of life
147
TETRALOGY OF FALLOT
What are the 4 clinical features?
- Large VSD
- Overriding aorta
- Sub pulmonary stenosis
- RVH
148
TETRALOGY OF FALLOT
Signs and symptoms
- Cyanotic
- Breathlessness
- Easy tiring
- Low sats
Associated with Down's and 22q deletions
149
TETRALOGY OF FALLOT
Investigations?
echo
CXR- small, up tilted, boot shaped apex
Harsh systolic murmur in LLSE
150
TETRALOGY OF FALLOT
Management?
Close VSD and relieve right ventricular outflow tract obstruction
- Shunt to increase pulmonary blood flow
151
What is Ebstein's Anomaly
Posterior leaflets of tricuspid valve displaced anteriorly
152
Cause of Ebsteins Anomaly?
Lithium in pregnancy (.eg.g bipolar mum)
153
What heart problems can develop due to Ebstein's Anomaly
Tricuspid regurg
Tricuspid Stenosis
RA enlargement
154
Murmur heard in tricuspid regurg?
Pan-systolic
155
Murmur heard in tricuspid stenosis?
Mid-diastolic
156
KAWASAKI'S DISEASE
What is it?
Idiopathic systemic vasculitis that most commonly effects children 6 months- 5 years
157
KAWASAKI'S DISEASE
Major complication?
Coronary artery aneurysm formation
158
KAWASAKI'S DISEASE
Clinical presentation?
MyHEART
Mucosal Involvement:-inflamed dry lips/ strawberry tongue
Hand and feet swelling
Eyes- bilateral conjunctivitis
lymphAdenopathy (cervical)
Rash
Temperature- >5 days of fever
159
KAWASAKI'S DISEASE
3 Phases?
```
Acute febrile (1-2 weeks)
Fever + 4 of criteria (MyHEART)
```
Subacute- remission of fever (4-6 weeks)
development of coronary artery aneurysms
Convalescent (6-12 weeks)
resolution of clinical signs + normalisation of inflammatory markers
160
KAWASAKI'S DISEASE
Ddx?
- Measles
- Rubella
- Parvovirus B19 infx
- Infectious Mononucleosis / Glandular Fever
- Scarlet fever
161
KAWASAKI'S DISEASE
Investigations?
```
Increased:
ESR&CRP
WCC
Platelets
AST
a1-antitrypsin
bilirubin
```
Echo is essential as it can reveal dilation and aneurysms of coronary arteries
162
KAWASAKI'S DISEASE
Treatment?
- Aspirin
- IV Immunoglobulins
(reduce the risk of aneurysms and thrombosis)
Echo 6 weeks later to check for aneurysms
163
KAWASAKI'S DISEASE
Tx for permanent inflammation?
Infliximab (anti-TNF)
164
HENOCH-SCHONLEIN PURPURA
What is it?
HSP is an IgA mediated, autoimmune hypersensitivity vasculitis of childhood
(Skin, joint, gut, kidneys)
165
HENOCH-SCHONLEIN PURPURA
Risk factors?
Infections ( group A strep, mycoplasma, EBV)
Vaccinations
Exposure to allergens, cold, pesticides
insect bite
166
HENOCH-SCHONLEIN PURPURA
Clinical presentation?
- Fever
- Rash
- Abdo pain/symptoms
- Renal involvement
- Arthritis
167
HENOCH-SCHONLEIN PURPURA
Diagnostic tests?
urinanalysis- protein/ haematuria
Raised ESR
raised serum IgA
Raised WCC
Anti-streptolysin O titrates increased (36%)- detect group A strep
168
HENOCH-SCHONLEIN PURPURA
Treatment?
NSAIDs for joint pain
Corticosteroids for arthralgia
Most recover in 2 months
169
INFECTIVE ENDOCARDITIS
risk factors?
IV drug users
prosthetics
structural heart defects
170
INFECTIVE ENDOCARDITIS
What is it?
Infection of valves/ endocardium, causing destruction due to infective organisms forming vegetation.
171
INFECTIVE ENDOCARDITIS
Symptoms?
- Fever
- Weight loss
- Night sweats
- Anaemia
172
INFECTIVE ENDOCARDITIS
Symptoms?
- Fever
- Weight loss
- Night sweats
- Anaemia
173
HENOCH-SCHONLEIN PURPURA
Complications?
massive GI bleeds
ileus
Haemoptysis (rare)
AKI (rare)
174
HENOCH-SCHONLEIN PURPURA
Classic Triad?
1) Purpura (purple spots/nodules not dissapearing on palpation)
2) Arthritis/ arthralgia (74%)
3) Abdominal pain (51%)
175
HENOCH-SCHONLEIN PURPURA
worries if purpura is non blanching?
Meningococcal disease
176
HENOCH-SCHONLEIN PURPURA
worries if purpura is non blanching?
Meningococcal disease
177
RHEUMATIC FEVER
What is it?
x
178
RHEUMATIC FEVER
What is it?
Systemic febrile ilness- reaction to group A B-haemolytic strep
+ Pharyngitis
179
RHEUMATIC FEVER
What is the Jones diagnostic criteria?
1/2 major + 2 minor plus evidence of preceding strep infection (scarlet fever/ throat swab/ high serum ASO titre)
180
RHEUMATIC FEVER
Major signs?
- Carditis(+ve echo, changed murmur, CCF, cardiomegaly)
- Polyarthritis
- Erythema marginatum
- Subcutaneous nodules
- Sydenham's chorea- neurological manifestation
181
RHEUMATIC FEVER
Minor signs?
- Fever
- ^ ESR/ CRP
- Arthralgia pain
- ECG PR interval <0.2s
- Previous rheumatic fever/ rheumatic heat disease
182
RHEUMATIC FEVER
Treatment?
Aspirin- careful of Reye's
Benzylpenicillin then Phenoxymethylpenicillin for Pharyngitis (sore throat)
Prednisolone may help
183
RHEUMATIC FEVER
What is given for Synderham's Chorea?
Prednisolone
consider haloperidol, carbemazepine, valproic acid
184
What is Haloperidol?
Anti-psychotic- used to treat psychotic disorders like Schizophrenia, Tourettes, severe behavioural problems in children
185
CHICKEN POX
What is it?
Highly infectious disease caused by varicella zoster virus (VZV)
186
CHICKEN POX
What does reactivation of VZV lead to?
Reactivation of dormant virus after a bout of chicken pox leads to herpes zoster (shingles) in posterior root ganglia
187
CHICKEN POX
Risk factors?
- Immunocompromised
- Older age
- Steroid use
- Malignancy
- Dangerous in neonates and to the foetus if contracted in pregnancy
188
CHICKEN POX
How long are you infectious for?
Infective from 4 days prior to rash until all lesions have scabbed. (day 5)
189
CHICKEN POX
Clinical presentation?
- temp 38-39
- Headache, malaise
- Crops of vesicles (itchy)
190
CHICKEN POX
Where are the vesicles normally found?
Mostly on the head, neck and trunk, very sparse on the limbs
191
CHICKEN POX
Cycle of a vesicle?
1) Macule
2) Papule
3) Vesicle (red surround)
4) Ulcers
5) Crust
192
CHICKEN POX
What does redness around the lesion suggest?
Bacterial superinfection
193
CHICKEN POX
Ddx?
Shingles- only one dermatome
(patient with vesicles at different stages of evolution in a one dermatome distribution)
- Generalised herpes zoster/ simplex
- Dermatitis herpetiformis
- Impetigo
194
CHICKEN POX
Diagnostic tests?
Clinical
| - Fluorescent antibody tests- test for IgM and IgG
195
CHICKEN POX
Complications?
Secondary bacterial infection of lesions
Pneumonia
Encephalitis
Disseminated haemorrhage chickenpox
Arthritis, nephritis, pancreatitis
196
CHICKEN POX
Treatment?
Calamine lotion
Antivaricella- zoister immunoglobulin &
Acyclovir (if severe) / at risk
Flucloxacillin in bacterial superinfection
- 5 days off school for kids
197
CHICKEN POX
Treatment?
Calamine lotion
Antivaricella- zoister immunoglobulin &
Acyclovir (if severe) / at risk
Flucloxacillin in bacterial superinfection
- 5 days off school for kids
198
MEASLES
How is it transmitted?
Respiratory droplets
incubation 7-12 days
199
MEASLES
What is it?
Acute viral infection caused by single stranded RNA morbillivirus from the paramyxovirus family
200
MEASLES
How long are you infectious for?
From prodrome until 4 days after the rash of measles appear
201
MEASLES
What is it?
NOTIFIABLE DISEASE
Acute viral infection caused by single stranded RNA morbillivirus from the paramyxovirus family
202
MEASLES
How long are you infectious for?
From prodrome until 4 days after the rash of measles appear
203
MEASLES
Cause?
RNA Morbillivirus from the paramyxovirus
204
MEASLES
Clinical presentation?
- Rash for at least 3 days
Fever for at least one day (often >40) and at least one of:
Cough
Coryza
Conjunctivitis
205
MEASLES
What is the prodrome made up of?
3-5 days of the 4 C's
- Cough
- Coryza
- Conjunctivitis
- Cranky
+ koplik's spots on palate - small, red spots each with a bluish- white speck in the centre
206
MEASLES
Describe the measles rash
First seen on forehead, neck and behind the ears and spreads to limb/trnk over 3-4 days
Fades after 3-4 days
Leaves behind brown discolouration
207
MEASLES
Ddx?
Rubella
Parvovirus B19
Enterovirus
- Scarlet fever
208
MEASLES
Diagnostic tests?
Lab diagnosis:
IgM & IgG positive
Salivary swab or serum sample for measles specific immunoglobulin taken within 6 weeks of onset
RNA detection in salivary swabs
209
MEASLES
Treatment?
Paracetamol/ Ibuprofen + fluids
210
MEASLES
Complications?
more common if <5 years or >20 years
- Otitis media
- Croup/ tracheitis
- Pneumonia
- Encephalitis
211
MEASLES
Complication of vitamin A deficiency?
Blindness
212
MEASLES
What is the most common cause of death in measles?
Pneumonia
213
MEASLES
What may be a complication in older patients?
Encephalitis
Pneumonia
214
What is subacute sclerosing panencephalitis?
7-13 years post measles: chronic complication
Progressive changes in behaviour, myoclonus, dystonia, dementia > death
215
MEASLES
Risks in pregnancy?
Increased risk of miscarriage, prematurity and low birthweight
216
SCARLET FEVER
What is it?
NOTIFIABLE DISEASE
- Endotoxin mediated disease arising from a specific bacterial infection by an erythrogenic toxin- producing strain of:
strep. pyogenes- Group A haemolytic streptococci
217
SCARLET FEVER
Epidemiology?
87% under 10 years old
Unusual under 2 years
218
SCARLET FEVER
Clinical presentation?
- Acute onset sore throat and fever then rash 24-48 hours after
- Strawberry tongue
- Scarletiniform rash:
Typically appears first on chest, axilla, behind ears
then trunk and legs after
Around mouth (circumoral)
219
SCARLET FEVER
Describe the rash?
Red, 'pin prick' blanching rash
| sandpaper/ rough- like
220
SCARLET FEVER
Describe the prodrome?
- Sore throat + Tonsillitis
- Fever
- Headache
- Vomiting and abdo pain
- Myalgia
221
SCARLET FEVER
Complications?
- Syndenhams Chorea
- Otitis media
- Rheumatic fever
- Glomerulonephritis
222
SCARLET FEVER
Ddx?
other viral exanthema
- Infectious mononucleosis (cause EBV)
- Toxic shock syndrome
- Kawasaki's
223
SCARLET FEVER
Diagnosis?
Clinical
Throat swab
Antigen detection kits
- Strep antibody tests
224
SCARLET FEVER
Treatment?
Penicillin/ Azithromycin for 10 days
Rest, fluids, para/ ibuprofen
225
RUBELLA
What is it?
Causative organism?
NOTIFIABLE DISEASE
An RNA virus (Rubivirus Togaviridae), transmitted as droplets, with an incubation period of 14-21 days
226
RUBELLA
How long are you infectious for?
Infectious for up to 5 days before and 5 days after start of rash
227
RUBELLA
Describe the prodrome?
- Lethargy
- Low grade fever
- Headache
- Mild conjunctivitis
- Anorexia
228
RUBELLA
Describe the rash?
Initially pink discrete macular rash that coalesce starting behind the ear and face, spreading the entire body
- Suboccipital lymphadenopathy
229
RUBELLA
Ddx?
- Contact dermatitis
- Erythema multiforme/drug allergy
- Measles
- Scarlet fever
- Kawasaki disease
230
RUBELLA
Diagnostic tests?
PCR testing
FBC shows low WBC with increased proportion of lymphocytes and thrombocytopenia
231
RUBELLA
Treatment?
Vaccine
| Antipyretics for fever
232
RUBELLA
What can infections during fetal development cause?
wks 1-4 = eye anomaly (70%)
wks 4-8 = cardiac abnormalities (40%)
wks 8-12 = deafness (30%)
233
SLAPPED CHEEK SYNDROME (Erythrovirus)
What is it?
Parvovirus B19 transmitted through droplets
234
SLAPPED CHEEK SYNDROME (Erythrovirus)
Prodrome symptoms?
(around 1 week)
- Mild
- Headache, rhinitis, sore throat, fever, malaise
THEN- 1 week of symptom free
235
SLAPPED CHEEK SYNDROME (Erythrovirus)
What happens after the 7-10 days of no symptoms after the prodrome?
Classic 'slapped cheek' rash
1-4 days after the facial rash, erythematous macular morbilliform rash develops on the limbs
- Arthralgia
236
SLAPPED CHEEK SYNDROME (Erythrovirus)
Ddx?
Rubella
Measles
Scarlet fever
EBV
237
SLAPPED CHEEK SYNDROME (Erythrovirus)
Diagnostic tests?
B19 specific IgM indicates current or recent infection
B19 specific IgG indicates immunity
PCR
238
cause of Impetigo?
staph aureus +/- strep pyogenes
239
What does Impetigo look like?
Well defined lesions starting around nose and face with honey/golden coloured crusts on erythematous base
240
Treatment to Impetigo?
Topical fusidic acid or oral flucloxacillin if severe
241
MENINGITIS
What is it?
When Micro-organisms reach the meninges by direct extension or by the bloodstream
242
MENINGITIS
Bacterial cause?
- Neisseria meningitidis
- Strep pnuemoniae
- Haemophilus influenza
GREAT KILLERS = give benzylpenicillin immediately
243
MENINGITIS
Viral cause?
```
Enterovirus
Mumps
HSV
HIV
EBV
```
244
MENINGITIS
Fungal cause?
Cryptococcus neoformans
Candida albicans
245
MENINGITIS
Risk factors?
- Immunocompromised
- Endocarditis
- DM
- Alcoholism and cirrhosis
- IV drug abuse
- Renal/adrenal insufficiency
- Malignancy
- SIckle cell disease
CROWDING
246
MENINGITIS
Pathophysiology?
Bacterial?
Viral?
BACTERIAL:
1) The pia-arachnoid is congested with polymorphs causing a layer of pus to form
VIRAL:
1) Lymphocytic inflammatory CSF without pus formation
2) No polymorphs/ adhesions
3) Little or no cerebral oedema unless encephalitis develops
247
MENINGITIS
Epidemilogy?
Common in infants, children or elderly
Viral meningitis> Bacterial
3,200 cases of bacterial per year
248
MENINGITIS
Septic signs?
(often before meningeal signs)
- Malaise, fever, headache, temperature, rigor, vomiting
- ^ HR/RR, reduced BP
- Poor feeding
- Abnormal cry
- PETECHIAL PURPURIC RASH (NON-BLANCHING)- dont expect this
249
MENINGITIS
Meningeal signs?
(comparatively late, less common in young children)
Neck stiffness
Photophobia
Bulging fontanelle
+ve Kernigs (resistance to extending knee when hip is flexed)
+ve Brudzinki's (neck flexion = hip flexion)
250
MENINGITIS
Ddx?
- Subarachnoid haemorrhage
- Migraine
- Intracranial mass lesion
251
MENINGITIS
Diagnostic tests?
- Lactate
- FBC
- Glucose
- U&Es
- Blood cultures
- Throat swabs
- Immediate lumbar puncture
252
MENINGITIS
Treatment?
Immediate IV Abx and blood/CSF cultures!
(Cefotaxime)
High flow O2
253
MENINGITIS
Treatment?
Immediate IV Abx and blood/CSF cultures!
(Cefotaxime)
High flow O2
Fluids if in shock
254
MENINGITIS
If this is suspected what must be given immediately to:
<3 months old?
>3 months old - 18years?
In GP before hospital?
<3 months= Cefotaxime + Amoxicillin
>3 months= Ceftriaxone + Dexamethasone
In GP = Benzylpenicillin (Cefotaxime if allergic)
255
COXSACKIE'S DISEASE (HAND FOOT AND MOUTH)
What is it?
Viral illness commonly causing lesions involving mouth hands and feet;
Transmitted by faecal-oral route
256
MENINGITIS
If this is suspected what must be given immediately to:
<3 months old?
>3 months old - 18years?
In GP before hospital?
<3 months= Cefotaxime + Amoxicillin
>3 months= Ceftriaxone + Dexamethasone
In GP = Benzylpenicillin (Cefotaxime if allergic)
257
COXSACKIE'S DISEASE (HAND FOOT AND MOUTH)
Aetiology?
Coxsackievirus A16
Enterovirus 71
258
COXSACKIE'S DISEASE (HAND FOOT AND MOUTH)
Epidemiology?
Common among infants younger than 10
outbreaks common in nurseries, schools and childcare centres
259
COXSACKIE'S DISEASE (HAND FOOT AND MOUTH)
Epidemiology?
Common among infants younger than 10
outbreaks common in nurseries, schools and childcare centres
260
COXSACKIE'S DISEASE (HAND FOOT AND MOUTH)
Prodrome?
- Fever
- Malaise
- Loss of appetite
- Sore mouth/throat
- Cough
- Abdo pain
261
COXSACKIE'S DISEASE (HAND FOOT AND MOUTH)
Describe the mouth lesions?
On buccal mucosa, tongue or hard palate
Begin as macular lesions that progress to vesicles which then erode
YELLOW ULCERS SURROUNDED BY RED HALOES
262
COXSACKIE'S DISEASE (HAND FOOT AND MOUTH)
Describe the skin lesions?
Palms, soles and between fingers/ toes
Erythematous macules but rapidly progress to grey vesicles with an erythematous base
Can also appear on trunk, thighs, buttocks, genitalia
263
COXSACKIE'S DISEASE (HAND FOOT AND MOUTH)
Ddx?
Herpes simplex/ zoster virus
Chicken pox
kawasakis disease
264
COXSACKIE'S DISEASE (HAND FOOT AND MOUTH)
Diagnostic tests?
Clinical diagnosis
swab of lesions
PCR
265
COXSACKIE'S DISEASE (HAND FOOT AND MOUTH)
Treatment?
Fluid intake
Soft diet
Para/ Ibuprofen
If mouth is very painful, use topical agents e,g, lidocaine oral gel
Stay off school until better
266
ENCEPHALITIS
Infective Causes?
- HSV
- Mumps
- Varicella zoster
- Rabies
- Parvovirus
- Immunocompromised
- Influenza
- TB
- Toxoplasmosis
- Malaria
267
ENCEPHALITIS
Clinical signs?
- Flu like prodrome
- Reduced consciousness
- Odd behaviour
- vomiting
- Fits/ seizures
- Raised temp
268
ENCEPHALITIS
Ix?
- CSF, MC&S, PCR
- Bloods
- Stool (enteroviruses)
- Urine
269
ENCEPHALITIS
Management?
HSE- Herpes simplex encephalitis= most treatable = Acyclovir
270
TUBERCULOSIS
When should you suspect?
IF:
Overseas contact
HIV +ve
Odd CXR
271
TUBERCULOSIS
Clinical signs
```
Anorexia
Low fever
failure to thrive
cough
malaise
```
272
TUBERCULOSIS
Diagnosis?
Tuberculin tests
Interferon gamma release assays/ Mantoux
Sputum culture + ziehl-neelson stain x3
CXR- consolidation, military spots (fine white dots on CXR- rare)
273
TUBERCULOSIS
Management?
Expert help;
Isoniazid (6 months)
Rifampicin (6 months)
Pyrazinamide (2 months)
Ethambutol (2 months)
274
TUBERCULOSIS
Tests?
Zeihl-Neeson Test with a Lowenstein Jenson culture medium
Mantoux test
Interferon gamma testing
275
TUBERCULOSIS
Complications?
1) Pleural effusion
2) Pericardial effusion
3) Lung collapse
4) Lung consolidation (becomes solid)
276
TUBERCULOSIS
Side effects of Isoniazid?
Rifampicin?
Isoniazid: neuropathy, low WCC, inhibits CYP450
Rifampicin: pink urine, low platelets, induces CYP450, Flu symptoms
277
TUBERCULOSIS
Side effects of Pyrazinamide?
Ethambutol?
Pyrazinamide: Rash/ arthralgia
Ethambutol: Optic Neuritis
278
TUBERCULOSIS
Tx of latent TB?
Isoniazid for 6 months
279
HIV
When should this be diagnosed in childhood?
AIM: Before 1st birthday
tests at 3&6 months:
HIV viral PCR
p24 antigen
Specific IgA
- Monitor CD4 count
280
HIV
When should you consider HIV in children?
- Unknown pyrexia (PUO)
- Parotid enlargement
- Lymphadenopathy
- Hepatosplenomegaly
- Slow to clear infections
- Persistent diarrhoea
- Shingles
- Reduced platelets
- with TB/ pneumocytosis
281
HIV
When should HAART begin
Those with AIDs defining conditions / CD4 <15%
282
TOXIC SHOCK SYNDROME
Characteristics?
- Fever >39 degrees
- Hypotension
- Diffuse erythematous, macular rash
283
TOXIC SHOCK SYNDROME
How is the toxin produced?
by s.aureus + group A strep
Toxin acts as a super antigen that can cause organ dysfunction, it can be released from infection at any site
284
TOXIC SHOCK SYNDROME
Management?
Intensive care
Infection areas- surgically debrided
Abx- Ceftriaxone + Clindamycin
285
BIRTH MARKS AND RASHES
What are strawberry marks?
Cavernous haemangioma
normally self limiting but beware over eye and in airway
286
BIRTH MARKS AND RASHES
What is a Port Wine Stain?
permanent capillary haemangioma
seen in Sturge-Weber Syndrome
287
BIRTH MARKS AND RASHES
What syndrome are moles common in? (Naevi)
Turner's
288
BIRTH MARKS AND RASHES
What are mongolian blue spots?
non- caucasian ancestry
Congential dermal melanocytosis
Commonly seen on lower back/ buttocks
289
BIRTH MARKS AND RASHES
What are cafe au lait spots?
flat light brown patches on the skin.
Over 5 = Neurofibromatosis
290
BIRTH MARKS AND RASHES
What are Milia (milk spots)?
sebaceous plugs from sweat glands
291
BIRTH MARKS AND RASHES
What are infantile urticaria?
erythema toxicum neonatorum-
Histamine reaction
Harmless red blotches- come and go in crops
292
Key points to remember in chickenpox?
beware in immunocompromised
293
Key points to remember in Measles?
Prodromal CCCK
- Cough
- Coryza
- Conjunctivitis
- Koplik spots (inside mouth)
294
Key points to remember in Parvovirus?
slapped cheek
295
Key points to remember in Coxsackie?
Hand, foot and mouth
296
Key points to remember in Mumps?
prodromal malaise
Parotids
can cause orchitis and infertility
297
What is Molluscum contagiosum?
pox like nodules that last for months, generally in axilla
298
What do you call a rash that accompanies a disease/ fever?
Exanthema
299
What rash is diabetes associated with
Acanthosis nigricans
300
Cause of Toxoplasmosis?
Infected cat faeces-
- Microcephaly
- Fits
- Sensorineural deafness
301
What infections are associated with Cat/Dog faeces?
Cat- Toxoplasmosis (fits, deafness)
Dog- Toxocara (acquired blindness)
302
What is Toxocara?
Infected associated with eating dog faeces
303
What is Urticaria?
'nettle-like' rashes, raised and itchy patches
allergen exposure,
linked to child abuse if late presentation, symmetry, odd history
304
TORCH
What is a TORCH infection and what does it stand for?
Infection to the developing foetus/ newborn by any of:
```
Toxoplasmosis
Other agents
Rubella (german measles)
Cytomegalovirus
Herpes simplex
```
305
TORCH
Signs of CMV?
- Growth retardation
- Microcephaly
- Hepatosplenomegaly
- Hepatitis
- Anemia/jaundice/ thrombocytopenia
306
TORCH
What is the most common congenital infection in the U.K?
Cytomegalovirus
CMV
307
TORCH
Signs of Rubella?
Sensorineural deafness
congenital cataracts + glaucoma
heart disease (PDA)
308
TORCH
Signs of Toxoplasmosis?
- Cerebral calcification
- Microcephaly/ hydrocephalus
- Chorioretinitis
- Cerebral palsy
309
How is Toxoplasmosis diagnosed, what is the cause and what is the treatment?
Diagnosed by serology >95% asymptomatic
Caused by raw meat/ cat faeces
Tx= Pyrimethamine + Sulphadiazine + Spiramycin
310
TORCH
GIve examples of 'other' infections?
- Syphillis
- Varicella- zoster
- Parvovirus B19
311
TORCH
Treatment of Syphillis?
Treatment of Herpes Simplex?
Benzylpenicillin
Acyclovir
312
TORCH
Signs of Herpes Simplex?
```
Blindness
Low IQ
epilepsy
Jaundice
Resp distress
30% die even when treated
```
313
ECZEMA
How common is it?
15-20% of children and is becoming more common
314
What is Eczema Herpeticum?
Severe infection of skin (HSV 1+2)
more commonly seen in kids with eczema, potentially life threatening = treat with Acyclovir
315
Causative drugs of Stevens- Johnson Syndrome?
Presentation?
- Sulfonamides
- Anti- epileptics
- Penicillin
- NSAIDs
Painful erythematous macules, severe mucosal ulceration
316
ANAPHYLAXIS
What is it and how is it diagnosed?
severe life threatening hypersensitivity reaction of sudden onset
ABC
317
ANAPHYLAXIS
What may bring on the IgE mediated reaction?
```
foods
insect sting
drugs
latex
exercise
inhaled allergens
```
318
ANAPHYLAXIS
Cause of fatal anaphylaxis' in adults?
Mainly nuts
319
ANAPHYLAXIS
Management?
ABCDE
```
IM Adrenaline
Under 6 months: 0.01mg
6 months to 6 years: 0.15mg
6-12 years: 0.3mg
Over 12 years: 0.5mg
```
Antihistamine
Hydrocortisone
Salbutamol if wheeze
high flow O2
IV fluids
Monitor pulse oximetry, ECG, BP
320
What is classed as low birth weight?
Extremely low?
Incredibly low?
<1500g
<1000g
<750g
ensure adequate resus once born
321
FAILURE TO THRIVE
Definition?
Failure to gain adequate weight or achieve adequate growth at the normal rate for age
322
What is the key determinant in questioning health of child?
Growth
323
FAILURE TO THRIVE
Most common cause?
Inadequate calorie intake:
Poor breastfeed technique
Poverty
Unsuitable food offered
Neglect
324
CHILDHOOD DEVELOPMENT
4 Domains of development?
- Gross motor
- Fine motor and vision
- Language and hearing
- Social
325
CHILDHOOD DEVELOPMENT
What is developmental surveillance?
Ongoing process of following child over time- can be incorporated into well-child checks, general physical examination, routine immunisation visits
326
CHILDHOOD DEVELOPMENT
Definition of Dev Delay?
Definition of Dev Disorder?
Dev. Delay- along normal route but takes longer to reach milestones
Dev. Disorder- does not follow normal pattern, impairment
327
CHILDHOOD DEVELOPMENT
Risk factors?
Biological:
- Prematurity
- Low birth weight
- birth asphyxia
- hearing/vision impairment
Environmental:
- poverty
- poor parental education
- maternal alcohol/drugs
328
CHILDHOOD DEVELOPMENT
What is the 'stepping' reflex and when should it be seen?
1) hold baby upright with one sole on table top
2) Hip and knee will flex and other foot step forward
(birth - variable [6 weeks])
329
CHILDHOOD DEVELOPMENT
What are the 'Palmar' and 'plantar' grasp reflexes and when should it be seen?
touch hand/ soles and baby will grasp / curl toes
palmar = until 6 months
plantar = until 9-12 months
330
CHILDHOOD DEVELOPMENT
What is the 'moro' reflex and when should it be seen?
1) hold baby supine and abruptly lower the body about 2 feet.
2) Arms will abduct/extend and legs flex
(until 2 months)
331
CHILDHOOD DEVELOPMENT
What is the 'tonic neck' reflex and when should it be seen?
1) baby supine, turn head to one side and hold jaw on shoulder
2) Arms/ legs the heads turned to will extend and opposite will flex
(until 6 months)
332
CHILDHOOD DEVELOPMENT
What is the 'sucking and rooting' reflex and when should it be seen?
1) stroke perioral skin at corner of the mouth
2) mouth will open and baby will turn head toward stimulus and suck
(until 4 months)
333
Name the primitive reflexes
Atonic neck reflex
Moro reflex
Landau reflex
Plantar grasp reflex
Palmar grasp reflex
Parachute reflex
Positive support reflex
Rooting reflex
Stepping reflex
Trunk incurvation
A M L PPPP R S T
334
CHILDHOOD DEVELOPMENT
What is the 'trunk incurvation'/ Galant's reflex and when should it be seen?
1) support baby prone and stroke one side of the back
2) spine will curve towards stimulated side
(0-2 months)
335
CHILDHOOD DEVELOPMENT
What is the 'Landau' reflex and when should it be seen?
1) suspend baby prone
2) head will lift up and the spine will straighten
(0-6 months)
336
CHILDHOOD DEVELOPMENT
What is the 'Parachute' reflex and when should it be seen?
1) suspend baby prone and slowly lower the head towards a surface
2) arms and legs will extend in a protective fashion
(8 months onwards)
337
CHILDHOOD DEVELOPMENT
What is the 'Positive support' reflex and when should it be seen?
1) hold baby upright until feet touch a surface
2) hips, knees and ankles will extend and partially bear weight for 20/30 seconds
(0-6 months)
338
What are the postural reflexes?
- Parachute
- Positive support
- Landau
- Neck/ head righting reflexes
- Lateral propping
339
What does persistence of primitive reflexes and lack of development of postural reflexes show?
Hallmark of motor neuron abnormality in the infant
340
Definition of childhood disability and 2 examples?
Physical or mental impairment preventing them going about their daily life
1) Down's
2) Cerebral Palsy
341
CEREBRAL PALSY
What is it?
Chronic disorder of movement and posture attributed to non-progressive disturbances that occurred in the fetal brain.
342
CEREBRAL PALSY
other problems as well as movement/posture?
- Cognition
- Communication
- Perception
- Sensation
- Behaviour
- Seizures
343
CEREBRAL PALSY
Antenatal causes?
80% antenatal e.g vascular occlusion/ congenital infection (CMV, toxo, rubella)
10% birth asphyxia/trauma
344
CEREBRAL PALSY
Postnatal causes?
- Meningitis/ encephalitis/ encephalopathy
- Head trauma
- Intraventricular haemorrhage
345
CEREBRAL PALSY
Clinical presentation?
(motor)
- Abnormal limb/trunk posture and tone
- Delayed motor milestones
- Abnormal gait
346
CEREBRAL PALSY
Clinical presentation?
(non-motor)
- feeding difficulties
- learning difficulties
- epilepsy
- language/speech difficulties
- persistent primitive reflexes
347
CEREBRAL PALSY
How is functional ability described/assessed?
Gross Motor Function Classification System (GMFCS)
348
CEREBRAL PALSY
What are the 5 levels of the GMFCS?
Level 1- walks without limitation
Level 2- walks with limitations
Level 3- walks using handheld mobility device
Level 4- self mobility with limitations may use powered mobility
Level 5- manual wheelchair transportation
349
CEREBRAL PALSY
Diagnosis?
Made on clinical presentation
MRI can identify cause but not routinely used for diagnosis
350
CEREBRAL PALSY
What are the 4 clinical subtypes?
- Spastic
- Dyskinetic
- Ataxic
- Mixed
351
CEREBRAL PALSY
Describe Spastic (90%)
- Damage to UMN pathway
- Limb tone persistently increased (spastic)
- Brisk deep tendon reflexes
- Clasp knife reflexes
352
CEREBRAL PALSY
Types of spastic?
Hemiplegia
Diplegia
Quadriplegia
353
CEREBRAL PALSY
What is quadriplegia associated with?
- Seizures
- Low IQ
- Swallowing difficulties
354
CEREBRAL PALSY
Decribe Dyskinetic
- Movements of involuntary control
- Muscle tone variable
Chorea
Athetosis
Dystonia
355
what is Chorea?
Jerky movements
356
what is Athetosis?
slow writhing movements distally eg fanning fingers
357
what is Dystonia?
twisting appearance from simultaneous contraction of agonist and antagonist muscle groups
358
CEREBRAL PALSY
What damage is associated with Ataxic CP
cerebellum damage
359
CEREBRAL PALSY
What damage is associated with Dyskinetic CP
basal ganglia damage
360
CEREBRAL PALSY
Describe Ataxic
- poor balance
- delayed motor development
- can be hypo/ hypertonia
- uncoordinated , intention tremor and ataxic gait (evident later on)
361
What is Ataxia?
Lack of voluntary co-ordination of muscle movements that can include ataxic gait, speech and abnormal eye movements
362
CEREBRAL PALSY
Tx for spasticity?
Oral Diazepam
Baclofen
Botulinum toxin (botox) for relaxing muscles
363
CEREBRAL PALSY
Professionals involved?
- Physiotherapist
- Speech Therapist
- Occupational Therapist
- Orthoptist/ Audiologist
- Dietitian
- Social worker
364
DOWN'S SYNDROME
What is it?
Trisomy 21
Cognitive impairment can be variable
365
DOWN'S SYNDROME
Risk factors?
Family Hx
Old maternal age
.40 increases risk of non-dysjunction
366
DOWN'S SYNDROME
Clinical presentation?
Cardio
GI
Eyes
Ears
Cardio Disorders (VSDs, Tetralogy of Fallot)
90% have hearing loss (sensorineural&conductive)
Eyes - Brushfield spots, cataracts, nystagmus, stabismus
GI- Atresias, Hirschsprungs, pyloric stenosis, Meckel's diverticulum
367
DOWN'S SYNDROME
Facial features?
- Epicanthic folds
- Protruding tongue
- Small low set ears
- Flat occiput + facial profile
- High arched palate
368
DOWN'S SYNDROME
Clinical features?
Orthopaedic
Endocrine
Neuro
Haemotological
Ortho- Hypotonia, short stature, single palmar crease, sandal gap deformity
Endo- hypothyroid
Neuro- learning difficulties, low IQ, Seizures, Dementia
Haem- 12x greater risk of infections due to impaired cellular immunity, increased risk of AML, ALL
Polycythaemia
369
EDWARD'S SYNDROME
What is it?
Trisomy 18
80% female
Severe psychomotor + growth retardation in those that survive 1st year of life
370
EDWARD'S SYNDROME
Skeletal abnormalities?
Typical hand feature
Radial/ Thumb aplasia
Short sternum- nipples look widely spread
Rocker bottom feet (flat)
Microcephaly/ microstomia
371
EDWARD'S SYNDROME
Craniofacial abnormalities?
- Odd low set ears
- Micrognathia (small jaw)
- Prominent occiput
372
EDWARD'S SYNDROME
What is the typical hand posture?
Fingers cannot be extended:
Index overriding middle finger
5th finger overriding 4th finger
373
PATAU'S SYNDROME
What is it?
Severe physical and mental congenital abnormalities due to
Trisomy 13
374
PATAU'S SYNDROME
Clinical presentation?
Congenital heart defects
IUGR and low BW
polydactyly and rocker bottom feet
Severe learning difficulties
Cleft lip palate
375
PATAU'S SYNDROME
What is Holoprosencephaly?
the brain doesnt divide into two halves
376
PATAU'S SYNDROME
Facial features?
Cleft lip and palate
Microphthalmia
Hypotelorism (reduced distance between eyes)
377
PATAU'S SYNDROME
Treatment?
Prognosis is BAD- average survival is 2.5 days.
50% live longer than one week
5-10% live longer than one year
378
Give examples of Genomic imprinting?
| pair of genes come from one parent
Angelmans
Prader Willi
379
Give an example of a Minosomy disorder?
Turner's syndrome
380
Give 5 examples of Trisomy disorders
Downs
Edwards
Pataus
XXX syndrome
Klinefelter's
381
Give examples of Autosomal Dominant conditions
Huntingtons
Neurofibromatosis
Hereditary spherocytosis
Marfans
382
Give examples of Autosomal recessive conditions
CF
Albinism
PKU (phenylketouria)
383
Give examples of X linked inheritance conditions
Duchenne's muscular dystrophy
Red-green colour blindness
G6PD deficiency
Haemophilia A/B
384
PRADER-WILLI SYNDROME
What is it?
First human disorder attributed to genomic imprinting
Characteristics
Infant: Hypotonia and development delay
Adolescence: Obesity, learning and behavioural difficulties (especially food)
385
PRADER-WILLI SYNDROME
Cause?
Deletion in the paternally inherited chromosome 15 or maternal uniparental disomy 15
OPPOSITE TO ANGELMAN'S
386
PRADER-WILLI SYNDROME
Clinical presentation?
(Infant)
- Hypotonia at birth
- Failure to thrive/ poor feeding
- Genital hypoplasia
- Delayed motor milestones
USUALLY blue eyes blonde hair
387
PRADER-WILLI SYNDROME
Clinical presentation?
(Childhood)
- Hyperphagia (always hungry)
Obesity (due to ^)
Short stature
Behavioural problems
Low IQ
388
PRADER-WILLI SYNDROME
Treatment?
Growth Hormone
Anti-psychotics- Olanzapine, haloperidol
Fluoxetine and SSRI's are sometimes efftective
389
ANGELMAN'S SYNDROME
What is it?
Genetic imprinting disorder due to maternal deletion of chromosome 15
OPPOSITE OF PRADER WILLI
390
PRADER-WILLI SYNDROME
Why do patients have hyperphagia?
Markedly elevated levels of ghrelin, hormone associated with hunger
391
ANGELMAN'S SYNDROME
Clinical presentation?
developmental delay
Motor milestones delayed
Speech impairment
behavioural signs
Ataxia (broad based gait)
Strabismus
Drooling
Fascination with water
Epilepsy 90%
Microcephaly
392
ANGELMAN'S SYNDROME
behavioural signs?
- Short attention span
- Laughter and happiness/ excited
- Laughs at most stimuli
- Hand flapping common
- Tendency to pinch/grab/bite
- Fascination with water
393
ANGELMAN'S SYNDROME
Facial features?
```
Microcephaly
flat occiput
Prominent mandible
wide mouth
wide spaced teeth
drooling/ tongue thrusting
```
394
ANGELMAN'S SYNDROME
Diagnosis?
Chromosomal analysis
v similar to autism
Fluorescence in situ hybridisation (FISH) is able to detect 80-85% of all deletions.
395
ANGELMAN'S SYNDROME
Treatment?
Behavioural modification programmes
speech therapy
physio therapy
Parental education
Anti-convulsants for Epilepsy- valproate/ clonazepam
396
TURNER'S SYNDROME
What is it?
Loss or abnormality of the second X chromosome
almost all affected infertile and many girls experience only short stature and ovarian failure
397
TURNER'S SYNDROME
What is there increased risk of?
Increased risk of CHD
Renal malformations
Hearing loss
osteoporosis
Obesity
Diabetes
Atherogenic lipid profile
398
TURNER'S SYNDROME
Clinical presentation of a Newborn?
Lymphoedema
- Cardiac/renal abnormalities
(coarctation, asbence of kidney)
399
TURNER'S SYNDROME
Clinical presentation of an infant?
- Short stature
- Webbed neck
- Broad chest/ widely spaced nipples
- Bicuspid aortic valve (15%)
- Coarctation of the aorta (10%)
- high arched palate
- Behavioural problems
- Recurrent otitis media/ hearing loss
400
TURNER'S SYNDROME
Clinical presentation of adolescence?
Gonodal dysgenisis:
Absent/incomplete puberty
Amenorrhoea
Impaired growth
401
NOONAN'S SYNDROME
What is it?
Autosomal dominant condition characterised by:
- CHD,
short stature,
broad/webbed neck,
sternal deformity,
developmental delay,
cryptochidism (1 testes drops)
increased bleeding tendency
characteristic facial features.
402
TURNER'S SYNDROME
Associated with what?
Autoimmune conditions:
Thyroid
Diabetes
Coeliac
Crohn's
403
TURNER'S SYNDROME
Diagnostic tests?
- Can be diagnosed by amniocentesis or chorionic villous sampling
- Chromosomal analysis
404
TURNER'S SYNDROME
Treatment?
treat complications and monitor autoimmune associations
SHORT STATURE: recombinant human growth hormone
Oestrogen (12 years) to initiate puberty and prevent osteoporosis
405
NOONAN'S SYNDROME
Cause?
Caused by mutations in the RAS/ MAPK pathway
406
NOONAN'S SYNDROME
Epidemiology?
Autosomal dominant
60% are new spontaneous mutations
407
NOONAN'S SYNDROME
Facial appearance?
wide, tall forehead
Hypertelorism
Ptosis/ down slanting eyes
Low set ears
408
NOONAN'S SYNDROME
MSK presentation?
short webbed neck
broad chest with widely spaced nipples
Pectus carinatum superiorly
Pectus excavatum inferiorly
short fingers and short stature
409
NOONAN'S SYNDROME
Appearance in general?
striking blue/green irises
curly or wooly hair
410
NOONAN'S SYNDROME
neuro/ haem/ cardiac/ feeding problems?
feeding and growth problems
Neuro: Learning disaility/ developmental delay
bleeding disorders with easy bruising/ bleeding
Cardiac: Pulmonary stenosis/ hypertrophic cardiomyopathy
411
NEUROFIBROMATOSIS
What is it?
Autosomal dominant disorder that encompass the rare diseases NF1, NF2 and Schwannomatosis
412
NEUROFIBROMATOSIS
What is NF1?
More common and caused by a defect to the NF1 gene- Skin lesions
413
NEUROFIBROMATOSIS
What is NF2?
Central form with CNS tumours rather than skin lesisons.
Inherited schwannomas, typically bilaterally, also meningiomas and ependymonas
414
NEUROFIBROMATOSIS
What is Schwannomatosis?
recently recognised form, characterised by multiple non cutaneous schwannomas which is a histologically benign nerve sheath tumour
415
NEUROFIBROMATOSIS
Epidemiology?
50% have no family Hx
416
NEUROFIBROMATOSIS
Clinical presentation of NF1?
Cafe-au-lait spots
Freckling in skin folds
Neurofibromas
Lisch nodules
Short stature and macrocephaly
417
NEUROFIBROMATOSIS
Complications of NF1?
Mild learning difficulties
Nerve root compression from neurofibromas
Increased risk of malignancy e.g. optic glioma
418
NEUROFIBROMATOSIS
Presentation of NF2?
45% have hearing problems
Schwannomas bilaterally, especially vestibular nerve (cranial/spinal)
Meningiomas/ ependymomas
presents generally in 20s
419
NEUROFIBROMATOSIS
Difference between NF1&NF2?
Rarely more than 6 cafe-au-lait spots in NF2, not as many as NF1.
420
NEUROFIBROMATOSIS
diagnostic criteria?
(NF1)
2 OR MORE OF:
1) 6+ cafe au lait spots (>5mm kids, >15mm older)
2) 2 or more neurofibromas
3) Freckling (skin folds)
4) Optic glioma
5) Lisch nodules (clumps of pigment in eyes)
6) Bone abnormalities ( sphenoid dysplasia- absence of bone around eyes)
7) Parent/sibling/child with NF1
421
NEUROFIBROMATOSIS
Treatment?
Intervene appropriately where tumours produce pressure symptoms or suggestive of malignant change
Possible surgery
422
FRAGILE X SYNDROME (MARTIN-BELL)
Cause?
FMR1 gene includes a CCG repeat.
As its passed from each generation it lengthens.
Once it reaches >200 no fragile X protein is made.
TRINUCLEOTIDE REPEAT DISORDER
423
FRAGILE X SYNDROME (MARTIN-BELL)
Clinical Signs?
Delayed speech + language
Delayed motor milestones secondary to hypotonia
Low IQ/ learning difficulties
hyperactivity/ emotional & behavioural problems
Autism
424
FRAGILE X SYNDROME (MARTIN-BELL)
Physical symptoms?
Long, narrow face
Large ears
Prominent jaw
Big testes
425
FRAGILE X SYNDROME (MARTIN-BELL)
Ix and Tx?
Molecular genetic testing of FMR1 gene
Minocycline = shows improvement in behaviour
426
KLINEFELTER SYNDROME
What is it?
47XXY
chief genetic cause of hypogonadism
427
KLINEFELTER SYNDROME
Clinical presentation?
Gyaecomastia
Infertility
taller than average
small testes/penis
Delayed/ absent puberty
less body hair, broader hips, longer legs
428
KLINEFELTER SYNDROME
Associations?
Psychosocial issues
Learning disability
Autoimmune disease
Osteoporosis
Decreased Sexual Maturation
429
KLINEFELTER SYNDROME
Management
Androgen therapy
mastectomy (gynaecomastia)
lifespan normal
Arm span may be longer than body length
430
Key Features of:
Patau Syndrome?
Microcephalic
small eyes
cleft lip/palate
scalp lesions
431
Key Features of:
Edward's Syndrome?
Micrognathia
Low set ears
Rocker bottom feet
Overlapping of fingers
432
Key Features of:
Fragile X Syndrome?
```
learning difficulties
macrocephaly
long face
large ears
Macro-orchidism
```
433
Key Features of:
Noonan Syndrome?
webbed neck
pectus excavatum
short stature
pulmonary stenosis
434
Key Features of:
Pierre- Robin Syndrome?
micrognathia
posterior displacement of the tongue (UAO)
cleft palate
435
Key Features of:
Prader- Willi Syndrome?
Hypotonia
Hypogonadism
Obesity/ Hyperphagia
436
Key Features of:
William's Syndrome?
short stature
learning difficulties
friendly, extrovert personality
transient neonatal hypercalcaemia
Supravalvular aortic stenosis
437
AUTISM
What is it?
neurodevelopmental disorder that includes a range of possible impairments in social interaction, repetitive behaviour and communication.
438
AUTISM
Definition?
the presence of abnormal or impaired development that is manifest before the age of 3.
439
AUTISM
Three characteristics of abnormal functioning?
1) Reciprocal social interaction
2) Impairment of language and communication
3) Restricted, repetitive behaviour
440
AUTISM
Male to female ratio?
3/4:1
75% male
441
AUTISM
Clinical presentation?
- Communication difficulties
- Social interaction
- Difficulties with imagination/ rigidity of thought
442
AUTISM
Examples of social interaction difficulties?
- no desire to interact with others
- oblivious to others feelings
- no understanding of unspoken social rules
- lack of empathy
- poor eye contact
443
AUTISM
Examples of imagination difficulties?
restrictive/ repetitive behaviours
obsessive fixations
inability to play/write imaginatively
same questions
444
AUTISM
Examples of communication difficulties?
repeats speech
disordered language
poor non-verbal communication
no social awareness, unable to start up or keep a conversation
445
AUTISM
Associated medical problems?
epilepsy 25-30%
visual and hearing impairment
mental health (ADHD, depression, anxiety)
446
AUTISM
Treatment?
Early behavioural intervention
education for parents
Support in schools
447
AUTISM
Treatment for Aggression/irritability?
Risperidone
448
AUTISM
Treatment for sleep difficulties?
Melatonin
449
AUTISM
Treatment for repetitive behaviours?
SSRI's
450
ASPERGER'S SYNDROME
What is it?
Pervasive development disorder which lies within the autistic spectrum.
451
ASPERGER'S SYNDROME
Ratio of boys to girls?
Boys 8:1 Girls
452
ASPERGER'S SYNDROME
Difference to autism?
- lack of delayed cognition and language,
- aspergers are above average intelligent
- Aspergers are more likely to seek social interaction and share activities/ friendships
453
ASPERGER'S SYNDROME
Clinical presentation?
- Obsessed with complex subjects
- concrete thinking
- Pedantic
- Normal speech
- Clumsiness
- Solitary but socially aware
- Poor sleep patterns
454
ADHD
What is the criteria?
1) Present before 12
2) developmentally inappropriate
3) Severel symptoms in multiple settings
4) clear effect on social/academic/ occupational functioning
455
ADHD
How many inattentive and hyperactive symptoms must be evident?
6/9 inattentive
6/9 hyperactive/impulsive symptoms
456
ADHD
Cause?
Prematurity
| Fetal alcohol syndrome
457
ADHD
3 core symptoms?
- Hyperactivity
- Inattention
- Impulsivity
458
ADHD
inattentive symptoms?
(9)
1) easily distracted
2) does not appear to be listening when spoken to
3) has difficulty sustaining attention
4) avoids/ dislikes sustained mental effort
5) forgetful in daily activities
6) difficulty following instructions/ fails to complete tasks
7) difficulty organising tasks
8) careless mistakes/ lack of attention to detail
9) loses important items
459
ADHD
Hyperactive/ impulsive symptoms?
(9)
Hyperactive:
1) squirms/ fidgets
2) cannot remain seated
3) runs/ climbs in inappropriate situations
4) often 'on the go'
5) talks excessively
6) cannot perform leisure activities quietly
Impulsive:
7) blurts out answers
8) has difficulty awaiting turn
9) interrupts / intrudes others
460
ADHD
Primary school child clinical picture?
- Distractibility
- restlessness
- disruptive behaviour
- low self esteem
- rejection by peers
- impaired family relations
461
ADHD
Adolescence clinical picture?
- difficulty in organisation
- persistent inattention
- aggressive anti-social behaviour
- alcohol and drug problems
- sexually inappropriate
- lack of achievement
462
ADHD
treatment?
1st: Methylphenidate (ritalin)- 6 week trial, monitor growth during tx
2nd- Atomoxetine
3rd- Lisdexamfetamine
(when unresponsive to maximum doses of other two treatments)
463
OCD
What are obsessions?
What are compulsions?
Obsessions are unwanted intrusive thoughts, images or urges that repeatedly enter the person's mind
Compulsions are repetitive behaviours or mental acts that the person feels drive to perform
464
OCD
Causes?
Neuro conditions
developmental factos (abuse)
Stressors/triggers (pregnancy)
465
OCD
Associated conditions?
- Depression
- Social and other phobias
- Alcohol misuse
- Generalised anxiety disorder
- Body dysmorphic disorder (BDD)
- Eating disorders - Schizophrenia
- Bipolar disorder
- Tourette’s
- Autism
466
OCD
clinical presentation?
Must be present on most days for at least two weeks
- not imposed by outside influences
- repetitive and unpleasant/ excessive
- interfere with individual functioning e.g. wasting time
467
OCD
Tx?
CBT
Exposure and repsonse prevention
SSRI's
468
DIFFERENCE BETWEEN SYNCOPE AND SEIZURE
Triggers?
Seizure:
Flashing lights/ hyperventilation
Syncope:
upright, exertion, blood, needles
469
DIFFERENCE BETWEEN SYNCOPE AND SEIZURE
Prodrome?
Seizure: Typical aura
Syncope:
nausea, sweating, palpitations
470
DIFFERENCE BETWEEN SYNCOPE AND SEIZURE
Duration?
Seizure: variable
Syncope: 1-30s
471
DIFFERENCE BETWEEN SYNCOPE AND SEIZURE
Convulsive jerks?
Seizure: Common prolonged
Syncope: less common and brief
472
DIFFERENCE BETWEEN SYNCOPE AND SEIZURE
incontinence?
Seizure: Common
Syncope: Uncommon
473
DIFFERENCE BETWEEN SYNCOPE AND SEIZURE
tongue bite?
Seizure: Common
Syncope: rare
474
DIFFERENCE BETWEEN SYNCOPE AND SEIZURE
colour?
Seizure:
Partial- pale
tonic clonic- red,blue
Syncope: very pale
475
DIFFERENCE BETWEEN SYNCOPE AND SEIZURE
post-ictal phase?
Seizure: yes, slow and confused
Syncope: no, rapid orientation
476
What is a seizure?
Transient occurence of signs/ symptoms due to abnormal excessive neuronal activity in the brain
477
what is syncope?
sudden reduction in cerebral perfusion with oxygenated blood
478
FEBRILE SEIZURES
What are they?
seizures occuring in children aged 6 months to 6 years with fever and raised temperature (>37.8)
479
FEBRILE SEIZURES
what is a simple febrile seizure?
- generalised tonic-clonic
- less than 15 mins
- typically do not recur within 24 hrs
- should be recovered/ drowsy within 1 hr
480
FEBRILE SEIZURES
what is a complex febrile seizure?
Focal features
may be recurrence within 24 hours
duration of more than 15 mins
481
How long is a febrile status epilepticus seizure?
longer than 30 mins
482
FEBRILE SEIZURES
Causes?
- metabolic (electrolytes/glucose)
- Viral (meningo)
- CNS lesion
- epilepsy
- trauma
483
FEBRILE SEIZURES
When should you urgently refer?
1) First febrile seizure
2) >5 min duration
3) Drowsy >1 hour after seizure
4) Previous history of:
- <18 months
- Complex seizure
- On Antibiotics
484
FEBRILE SEIZURES
when should a LP be considered?
In children up to 18 months
485
Contra- indications of LP?
- reduced consciousness
- septicaemic shock
- likely invasie meningococcal
- signs of raised ICP
- focal neurology
- bleeding tendency
486
REFLEX ANOXIC SEIZURE
What is it?
Paroxysmal, self limiting brief asystole (<15s) that occurs in infants (6 months- 2 yrs) due to common triggers such as:
- Pain
- cold foods
- Fright
- Fever
487
treatment of seizures?
Diazepam/ Midazolam, Lorazepam
if lasting >5 min
488
REFLEX ANOXIC SEIZURE
Clinical presentation?
stops breathing
stiff/rigidity
incontinence
pale/blue
- NO tongue bite
489
REFLEX ANOXIC SEIZURE
Pathophysiology?
Cardiac asystole from vagal inhibition, diagnosis through vagal excitation tests
490
REFLEX ANOXIC SEIZURE
Tx?
Check ferritin
No drugs needed
pacemaker might be a option, child usually grows out of it
491
EPILEPSY
What is it?
Chronic neurological disorder characterised by recurrent unprovoked seizures
492
EPILEPSY
2 broad classifications?
Generalised (both hemispheres)
Focal/Partial
493
EPILEPSY
Describe absent epilepsy?
1) transient LoC for no more than 30s
2) Abrupt onset and no motor phenomena apart from eyelid flickering
3) Often precipitated by hyperventilation
4) Stare momentarily and stop moving
5) No recall but knows theyve missed something
6) 2/3rds female
7) 4-12 yrs old
494
EPILEPSY
types of generalised seizures?
```
Absent
Myoclonic
Tonic
Tonic-clonic
Atonic
```
495
EPILEPSY
Types of partial/focal seizures?
Frontal
Temporal
Parietal
Occipital
496
EPILEPSY
Describe myoclonic seizure?
Brief, repetitive jerking movements of limbs, neck or trunk
497
What is a hiccough/ hiccups?
non epileptic myoclonic seizure of diaphragm
498
EPILEPSY
Describe a tonic seizure?
Generalised increase in tone
499
EPILEPSY
Describe Tonic- clonic seizures?
(tonic phase)
- Rigid
- Fall to ground
- Stop breathing/ cyanosis
500
EPILEPSY
Describe Tonic- clonic seizures?
(clonic phase)
- Rhythmic contractions
- Limb jerking
- irregular breathing
- Saliva/ cyanosis
- tongue bite/incontinence
501
EPILEPSY
what follows a tonic-clonic seizure and how long do they last?
Followed by deep sleep/ unconsciousness for up to several hours
Seizure lasts seconds to minutes
502
EPILEPSY
Describe an atonic seizure
Transient loss of muscle tone causing drop to the floor or drop of head
503
EPILEPSY
Describe a frontal seizure
motor phenomena (clonic)
Assymetrical
504
EPILEPSY
Describe a parietal seizure
Contralateral altered sensation
505
EPILEPSY
Describe a temporal seizure
- Auditory/ smell/ taste phenomena
- Lip smacking/ plucking clothes
- Longer seizures
- Deja vu
506
EPILEPSY
Describe a occipital seizure
Vision distortion
507
EPILEPSY
Causes?
Usually none found
Infection
Hypo (glucose, Na, Ca, Mg)
Trauma
Metabolic defects
CNS tumour
Lights/ exercise
508
EPILEPSY
Ix?
EEG
History diagnosis
CT/MRI
509
EPILEPSY
Management of generalised?
Trigger education
1st line- Valoproate/ carbamazepine
2nd line- Lamotrigine
510
EPILEPSY
Management of focal?
1st line- Valproate/ carbamazepine/ lamotrigine
2nd line- Topiramate, gabepentin, tigabine, bigatrin
511
EPILEPSY
Management of prolonged seizures?
Rectal diazepam
512
STATUS EPILEPTICUS
Management?
ABC
Supportive therapy:
Secure airway
IV access
check temp
Check glucose
Drugs:
Lorazepam IV (5 min)
Lorazepam IV (15 min)
Phenytoin IV (20 min)
Refer to PICU
513
INFANTILE SPASMS/ WEST SYNDROME
3 components?
1) infantile spasms
2) hypsarrhythmia
3) General learning disability
514
INFANTILE SPASMS/ WEST SYNDROME
Epidemiology?
Peak incidence around 4-7 months
Confined to infants and v small children
515
INFANTILE SPASMS/ WEST SYNDROME
Clinical presentation?
Describe the spasms?
Spasms
Learning disability (70-90%)
Hypopigmented skin lesions
Mild/Moderate growth restriction
516
INFANTILE SPASMS/ WEST SYNDROME
Describe the spasms?
- Clusters of head nodding/jerks
Sudden rapid tonic contraction of the trunk and limb muscles
- last between 5-10 seconds
Occur just before/ after sleep
517
INFANTILE SPASMS/ WEST SYNDROME
Diagnosis?
EEG shows hypsarrhythmia which is crucial to diagnosis
518
INFANTILE SPASMS/ WEST SYNDROME
treatment?
Vigabatrin (50% success)
ACTH (50-65% success) {daily IM injections}
Prednisolone
PROGNOSIS IS POOR
519
TUBEROUS SCLEROSIS
What is it?
Multi-system
Formation of hamartomas in many organs, commonly the brain, skin and kidneys, which account for many of the clinical symptoms
520
TUBEROUS SCLEROSIS
Cause?
Mutation of TSC1 or TSC2 gene on chromosome 9 or 16
521
TUBEROUS SCLEROSIS
How does the affected gene cause the defect?
Hamartin and tuberin which form a regulatory complex responsible for limiting the activity of an important intracellular regulator of cell growth and metabolism
522
TUBEROUS SCLEROSIS
Epidemiology?
1 in 5,800
Autosomal dominant
TSC2 mutations are associated with more severe disease
523
TUBEROUS SCLEROSIS
Clinical presentation?
Epilepsy/ neuro
Behavioural problems
Skin/teeth involvement
CKD
524
TUBEROUS SCLEROSIS
Epilepsy seen?
Focal seizures and infantile spasms occur in infancy secondary to tuber formation in the brain
525
TUBEROUS SCLEROSIS
Cutaneous Features?
depigmented 'ash-leaf' spots which fluoresce under UV light
roughened patches of skin over lumbar spine (Shagreen patches)
adenoma sebaceum (angiofibromas): butterfly distribution over nose
fibromata beneath nails (subungual fibromata)
526
TUBEROUS SCLEROSIS
Behavioural problems?
Learning difficulties
Social deficits
Receptive/ expressive language deficits
Attention deficit
527
TUBEROUS SCLEROSIS
Diagnosis?
Clinical
EEG- epilepsy
MRI head- cortical tubers
528
TUBEROUS SCLEROSIS
Tx?
Treat complications
Vigabatrin is 1st
529
MUSCULAR DYSTROPHY
What type of genetic defect is it?
X-linked recessive
Mutation to gene encoding dystrophin
530
what is dystrophin?
Part of a large membrane associated protein in muscle which connects the muscle membrane to actin
531
MUSCULAR DYSTROPHY
Types?
which is more severe?
Duchenne- loss of Dystrophin
Becker - misshapen dystrophin
- Duchenne is more severe as 1 or both of the binding sites are lost (frameshift mutation) (No dystrophin)
532
MUSCULAR DYSTROPHY
Clinical presentation?
- Waddling clumsy gait
- Calf pseudohypertrophy
- Boys aged 1-6 for Duchenne and 10-20 for Becker
- Classic gower manoeuvre
- resp impairment
- Wheelchair needed 9-12 years
- Scoliosis
- osteoporosis
533
MUSCULAR DYSTROPHY
Diagnostic tests?
Creatinine kinase RAISED
Muscle biopsy
Abnormal fibres surrounded fat/fibrous tissue
534
MUSCULAR DYSTROPHY
Treatment?
Appropriate exercise helps to maintain muscle power and mobility and delays the onset of scoliosis
PREDNISOLONE- slows the decline in muscle strength and function in the short term
535
MUSCULAR DYSTROPHY
What is the Gower Manoeuvre?
Using hands to crawl up from a sitting position to a standing position
536
GASTROENTERITIS
Common cause in children and adults?
Children= Rotavirus
Adults = Noravirus
537
GASTROENTERITIS
how many deaths of children per year due to rotavirus?
600k
Vaccine is part of the schedule
538
GASTROENTERITIS
Risk factors?
Poor hygiene
Immunocompromised
Poorly cooked food
539
GASTROENTERITIS
Clinical presentation?
tests?
Sudden onset D+V
Pain
Stool sample test
540
GASTROENTERITIS
complications?
- Dehydration
- Malnutrition
- RARE temporary intolerance to sugar following D+V
541
GASTROENTERITIS
management?
Correct dehydration
Start Oral Rehydration therapy (Dioralyte)
IV for those in shock
542
GASTROENTERITIS
Common complication?
transient lactose intolerance
remove lactose from diet and then slowly reintroduce after a few months
543
what is colic?
when an infant who isn't sick or hungry cries for more than 3 hours a day, more than 3 days a week, for more than 3 weeks
544
COWS MILK ALLERGY (CMPI)
What immunoglobulin is it associated to
IgG & IgE
545
COWS MILK ALLERGY (CMPI)
How is it confirmed?
RAST test- skin prick blood test to measure IgE specific antibodies in the blood
546
COWS MILK ALLERGY (CMPI)
management?
When is it normally resolved by?
What to do in severe/ allergic reactions?
Avoid Cows milk
Normally resolved by 5 years old
Adrenaline in severe reactions
Antihistamines for allergic
547
Signs of Post Infective lactose Intolerance and how is it confirmed?
- weight loss
- stools green and frothy
- Increased frequency
Confirmed by presence of non-absorbed sugars in the stool
548
How to reduce toddlers diarrhoea and what may be the underlying cause?
Potential intestinal motility delay
reduced by adequate fat/fibre to slow motility
Presence of mucous and undigested vegetables common
549
COELIAC DISEASE
What is it?
Gluten sensitive enteropathy- damaging immunological response to proximal small intestine mucosa
550
COELIAC DISEASE
genetic association?
Linked to HLA DQ2 and less so to DQ8
551
COELIAC DISEASE
Risks?
Downs
T1DM
Autoimmune thyroid disease
552
COELIAC DISEASE
gastro clinical presentation?
Profound malabsorption
foul smelling diarrhoea
Abdo distension
weight loss
failure to thrive/ anaemia/low folate and ferritin
553
COELIAC DISEASE
Non- GI manisfestations?
Dermatitis herpetiformis
Severe pruritis
osteoporosis
Dental enamel defects
macules/ papules/ vesicles
554
COELIAC DISEASE
diagnosis?
IgA TTG antibodies and endomysial antibodies
small intestinal biopsy
555
COELIAC DISEASE
What does small biopsy show?
1) Villous atrophy
2) Intraepithelial lymphocytes
3) Crypt hypertrophy
556
CROHN'S
what is it?
Transmural chronic inflammatory disease affecting distal ileum and proximal colon (ileocaecal)
- Fistulae may develop between loops of bowel, skin, or other organs
557
CROHN'S
Presentation?
Triad:
Weight loss
Bad smell diarrhoea
Abdominal pain
Nausea/vomiting
Fever
Urgency
growth delay
558
CROHN'S
Non-intestinal manifestations?
Oral lesions
Clubbing
Uveitis (middle layer of eye)
Arthralgia (UC too)
Erythema nodosum (UC too)
559
CROHN'S
What is Erythema Nodosum
Erythema nodosum is a type of skin inflammation that is located in a part of the fatty layer of skin.
Erythema nodosum results in reddish, painful, tender lumps most commonly located in the front of the legs below the knees
560
CROHN'S
Diagnosis?
Exclude infection with stool MC&S
Colonoscopy with biopsy
561
CROHN'S
histological findings?
- non caseating, epithelioid cell granulomata
562
CROHN'S
Endoscopic findings?
small bowel narrowing, fissuring, and bowel wall thickened
Skip lesions
563
Management?
Flare ups- Steroids
Immunosuppression- Methotrexate/ Azathioprine
Anti- TNF biologics
564
ULCERATIVE COLITIS
What is it?
Recurrent ulcerating disease involving mucosa of the colon (mainly distal colon)
565
ULCERATIVE COLITIS
Presentation?
Rectal bleeding
bloody diarrhoea
Colicky pain
weight loss
growth failure
566
ULCERATIVE COLITIS
Diagnosis?
Upper and ileocolonscopy
Barium swallow to rule out Crohn's
never further than ileocaecal valve
goblet cell depletion
crypt abscesses
567
ULCERATIVE COLITIS
histology?
ulceration
Crypt damage
mucosal inflammation
568
ULCERATIVE COLITIS
management?
5-ASA's
sulfasalazine/ Mesalazine/ Balsalazide
steroids for widespread exacerbations
Azathioprine for induction of remission + low dose corticosteroid
569
Difference between Crohn's and UC?
Crohn's vs UC
Transmural vs Mucosal
Granulomas vs polymorphonuclear aggregation
Skip lesions vs continuous
Whole gut vs colon
goblet cells vs reduced goblet cells
Strictures/fistula/abscess vs presence of crypts (pseudopolyps as cells try to regenerate)
570
What is Kwashiorkor malnutrition?
Due to low intake of protein + essential amino acids but has enough energy from other sources
- Oedema
- Hair changes
- Mental changes
571
What is Marasmus?
Malnutrition due to lack of calories from all energy sources
572
What is Intussusception?
Most common cause of intestinal obstruction, one segment of the bowel invaginate the other.
573
where is Intussusception likely to occur?
Ileocaecal
574
Clinical presentation of Intussusception?
Sudden onset
paroxysms of colicky abdominal pain
Early bile stained vomit
RUQ mass (sausage shape)
RED CURRENT JELLY stools
575
PYLORIC STENOSIS
Presentation?
- Projectile vomiting
- No bile
- Constipation
576
PYLORIC STENOSIS
Metabolic signs?
Metabolic alkalosis
- Hyponatraemia
- Hypochloraemia
- Hypokalaemia
577
PYLORIC STENOSIS
Signs on examination?
Olive sized pyloric mass
Clearly visible LUQ peristalsis
578
PYLORIC STENOSIS
Management?
Ramstedt's Pyloromyotomy
579
NECROTISING ENTEROCOLITIS (NEC)
What is it?
Inflammatory bowel necrosis, most common GI emergency in neonates
(tends to affect premature)
580
NECROTISING ENTEROCOLITIS (NEC)
Risk factors?
- Prematurity
- Low BW
- PDA
581
NECROTISING ENTEROCOLITIS (NEC)
Clinical presentation?
- Feeding problems with abdominal distension and vomiting
Bloody mucoid stool and bilious vomiting
582
NECROTISING ENTEROCOLITIS (NEC)
Diagnosis?
Abdo X-ray
- Pneumatosis intestinalis (gas in gut wall)
Portal venous gas
583
NECROTISING ENTEROCOLITIS (NEC)
Tx/
Nil by mouth
IV Cefotaxime/ Vancomycin
for 10-14 days
584
HIRSCHPRUNG'S DISEASE
What is it?
No bowel movements due to missing nerve cells (ganglia) in segment of
baby’s colon
585
HIRSCHPRUNG'S DISEASE
Presentation?
- No bowel movements in 1st 48hrs = Meconium Ileus
- Vomiting bilious green
- Constipation
586
HIRSCHPRUNG'S DISEASE
Complications?
GI perforation
| Short gut syndrome after surgery
587
HIRSCHPRUNG'S DISEASE
Diagnosis?
X ray with barium contrast
Rectal biopsy
588
HIRSCHPRUNG'S DISEASE
Management?
Bowel washouts/irrigation
Bypass surgery/ ileostomy//colostomy
589
JAUNDICE
Why do neonates have higher bilirubin levels than adults?
1) higher concentration of RBCs with short lifespan
2) RBCs breakdown = unconjugated bilirubin
3) unconjugated bilirubin becomes conjugated in liver = liver immaturity = slower conjugation
590
JAUNDICE
What may be the possible cause of jaundice in neonates at:
<24 hours?
>14 days?
<24hrs = Rhesus haemolytic disease (+ve Coombs test)
>14 days = Biliary atresia (pale stools)
591
JAUNDICE
Risk factors?
- Low BW
- Prematurity
- Breast fed babies
- Maternal diabetes
- Family Hx
592
JAUNDICE
Clinical presentation?
First seen normally on forehead and face
Neuro signs:
- change in muscle tone, altered crying
- Pale stools and dark urine
593
JAUNDICE
What is the name of continued high levels of jaundice?
Kernicterus
594
JAUNDICE
Treatment of jaundice?
Phototherapy
Exchange transfusion
595
JAUNDICE
What is Kernicterus?
Acute bilirubin encephalopathy that can cause:
Athetoid movements
Deafness
Low IQ
(brain damage)
prevented by jaundice treatments
596
GORD
Signs?
How is it diagnosed?
Regurgitation
Distress after feeds / failure to feed & thrive
Apnoea
Diagnosed by endoscopy if required
597
GORD
Management?
1) Avoid over-feeding
2) Thicken feeds
3) Antacid + Na/Mg alginate (Gaviscon)
4) Introduce PPI
598
GORD
If GORD does not resolve in 6-9 months what can be done?
Fundoplication if still failure to thrive / severe oesophagitis
599
What is Meckel's Diverticulum?
What is the Rule of 2's?
most frequent malformation of GI tract
```
2% of population
2 feet from ileocaecal valve
2 different mucosa
common in less than 2 years old
Males 2x than females
2 inches long or less
```
Meckel diverticulum occurs in 2% of the population, 2% are symptomatic, children are usually less than 2 years, affects males twice as often as females, is located 2 feet proximal to the ileocecal valve, is 2 inches long or less, and can have 2 types of the mucosal lining.
600
How is Meckel's Diverticulum diagnosed?
Always considered when patients have haemorrhage or obstruction
CT scan if volvulus/intusseception
601
When should a Diverticula be resected?
>2cm
narrow neck
fibrous band to abdominal wall
inflamed/ thickened
602
what can congenital diaphragmatic hernia lead to?
Impaired lung development
- Pulmonary hypoplasia
- Pulmonary hypertension
603
What is Gastroschisis?
Evisceration of abdominal contents, opening usually >5cm
604
Signs of biliary atresia?
Treatment?
Elevated unconjugated/conjugated bilrubin = Jaundice
Pale stools/ dark urine
kasai procedure
605
Causes of increased interstitial fluid?
o Lymph drainage – lymphoedema (eg congenital, blockade)
o Venous pressure and drainage – venous obstruction eg
thrombosis
o Lowered oncotic pressure (low albumin / protein)
▪ Malnutrition
▪ Decreased production from liver
▪ Increased loss eg gut, Kidney (nephrotic syndrome)
o Salt and Water retention
▪ Heart failure
▪ Kidney – impaired GFR
606
Causes of Oedema (renal)
o Glomerulonephritis (Nephrotic syndrome, diabetes, HSP, SLE, infection, malignancy, Alports)
o orthostatic proteinuria, reduced renal mass, hypertension
o tubular proteinuria (ATN, PKD)
607
NEPHROTIC SYNDROME
most common cause?
minimal change disease
608
NEPHROTIC SYNDROME
Triad?
o Heavy proteinuria
▪ First morning urine (protein:creatinine)
• >1g/m2/24 hrs
o Hypoalbuminaemia
▪ Normal range 35-45g/l
▪ Fluid retention and oedema normally <25-30 but not cut
off
o Oedema
▪ Pitting oedema and gravitational
o Can also have Hyperlipidaemia
609
NEPHROTIC SYNDROME
Investigations?
Urinalysis (dipstick = UTI, MC&S = protein)
BP
Low serum albumin
Low Anti- T III = prone to renal vein thrombosis/ DVT/ PE
- Antistreptolysin O and anti-DNAse B titres and throat swab
610
NEPHROTIC SYNDROME
3 types?
Congenital (<1 year)
Steroid sensitive
steroid resistant
611
NEPHROTIC SYNDROME
Signs of Steroid sensitive NS?
1) Normal BP, renal function
2) no macroscopic haematuria
3) no features to suggest nephritis
4) Normally minimal change histologically
612
NEPHROTIC SYNDROME
Signs of Steroid resistant NS?
1) Elevated BP, impaired renal function
2) haematuria
3) features to suggest nephritis
4) underlying glomerulopathy/ basement membrane abnormality histologically
613
NEPHROTIC SYNDROME
Tx of Steroid sensitive NS?
1st - Prednisolone
2nd - renal biopsy if unresponsive
614
NEPHROTIC SYNDROME
Tx of Steroid resistant NS?
Diuretics/ACE-I = To treat oedema
NSAIDs may reduce proteinuria
615
NEPHRITIC SYNDROME
Causes?
Post infection - Grp A b-haemolytic strep
HSP vasculitis
IgA nephropathy
616
Clinical features of Acute Glomerulonephritis?
Haematuria
Proteinuria
Decreased urine output (salt and water retention = HTN)
Impaired GFR (Increased Creatinine)
617
Investigations of Acute Glomerulonephritis?
o FBC – mild normochromic, normocytic anaemia
o U&Es – increased urea and creatinine (hyperkalaemia, acidosis)
o Low C3 + normal C4 complement
o Urinalysis
▪ Macroscopic haematuria
▪ Dipstick – protein (albumin): creatinine ratio showing proteinuria
▪ Microscopy – RBC casts
618
Management of Acute Glomerulonephritis?
o Fluid balance (salt restriction + diuretics)
o Treat HTN
o Correct electrolyte imbalance (potassium/ acidosis)
o Penicillin if infection
619
What is Alport Syndrome?
X linked recessive disorder associated with hearing loss, ocular defects and progressive kidney disease
Almost always have haematuria
620
UTI
give an example of an upper and lower
Upper = Pyelonephritis
Lower = Cystitis
621
UTI
Most common organisms?
E.coli
Klebsiella
622
UTI
Presentation of Upper/Lower UTI?
Upper:
- Fever
- Loin pain
- failure to thrive, jaundice
Lower:
- Dysuria
- Urinary frequency
- Incontinence
- Lower abdominal pain
- Haematuria
623
UTI
What could dysuria alone mean?
Vulvitis in girls
Balanitis in boys
624
UTI
Diagnosis and Investigations?
Urinalysis:
MC&S
Dipstick (leucocytes and nitrites)
625
UTI
Management?
<3months - IV Amoxicillin and Gentamycin
>3 months - Trimethoprim/ Nitrofurantoin/ Amoxicillin
626
What is Vesicoureteric reflux?
Anomaly of vesicoureteric junction where the ureters are displaced laterally and enter bladder directly = not at an angle means back flow
627
What can VUR cause?
How is VUR diagnosed?
incomplete emptying = increase risk of UTI (cystitis)
Renal damage if high pressure / infrarenal reflux
Dx = micturating cystourethrogram
628
AKI
Pre renal Causes?
- Hypovolaemia (nephrotic syndrome, gastroenteritis, haemorrhage)
- circulatory failure
629
AKI
Renal causes?
Vascular- haemolytic uraemia syndrome/ vasculitis
tubular - Acute tubular necrosis
Glomerular - glomerulonephritis
Interstital - pyelonephritis
630
AKI
Post renal causes?
Obstruction
congenital- posterior urethral valves
acquired- blocked urinary catheter
631
What is AKI?
Acute kidney injury characterised by rapid rise in creatinine and development of oliguria/anuria.
632
Secondary causes of AKI?
Cardiac surgery
Bone marrow transplant
Toxicity - DRUGS (NSAIDs, vancomycin, acyclovir, aminoglycosides)
633
AKI
Signs on bloods?
```
High:
K
Creatinine
Urea
maybe phosphate
```
Low:
Ca
Na
Cl
634
AKI
management?
- Fluids
- If urine osmolality low = furosemide
Renal replacement therapy = pulmonary oedema/HTN/ not responding to tx
635
Management of Hyperkalaemia?
IV calcium gluconate (protect cardiac membrane)
Nebulised salbutamol
Diuretics/Dialysis to remove K from body
636
CKD
Causes?
- Congenital dysplastic kidney
- pyelonephritis
- glomerulonephritis
- recurrent infection
- reflux nephropathy
- AKI - leading to necrosis
637
CKD
Investigations?
- Monitor growth
- BP-
- U+E
- Ca2+ (often low), Phosphate (often high)
638
CKD
Presentation?
- Anorexia/lethargy
- Polydipsia/polyuria
- ^BP, hypertensive, retinopathy
- anaemia
- Failure to thrive
- seizures
- renal Ricketts
639
Management of CKD?
Treat Anaemia, acidosis
Manage diet
Prevent renal osteodystrophy
Dialysis + transplant
640
Ddx of Proteinuria?
- Orthostatic proteinuria
- Glomerular abnormalities
o Minimal change disease - nephrotic syndrome
o Glomerulonephritis (nephrotic syndrome)
```
o DM
- Increased glomerular filtration pressure
- Reduced renal mass
- Hypertension
- Tubular proteinuria
o ATN
o PKD
o Pyelonephritis
```
641
Ddx of Haematuria?
Non glomerular:
- Infection
- Trauma to genitalia/ kidney
- stones
- tumour
- sickle cell
- renal vein thrombosis
```
Glomerular:
- Vasculitis
- SLE
- Post infectious
- Glomerulonephritis
IgA nephropathy
```
Alports syndrome
Goodpastures
642
DIABETES
Cause of T1 DM?
T-cell mediated B-cell destruction in the Islets of Langerhan causing no insulin production
643
DIABETES
What % of childhood diabetes is type 1?
97%
644
DIABETES
Insulin actions?
1) Stimulates formation of glycogen from glucose in the liver
2) Stimulates glucose uptake form blood into cells
3) Lowers blood sugar
645
DIABETES
What is the threshold for:
Fasting glucose
OGTT
HbA1c
Fasting = >7mmol/L
OGTT = >11.1mmol/L
HbA1c = >6.5%
646
DIABETES
How does the body respond when insulin is not working efficiently?
Gluconeogenesis = produce glucose from amino acids / muscle breakdown
Ketogenesis = fatty acids converted to ketones
647
DIABETES
Clinical presentation?
- Polyuria
- Polydipsia
- Weight loss
- Lethargy
- Poor growth
648
Signs of DKA?
- Vomiting
- Acidosis
- Reduced consciousness
- Ketonuria
649
DKA on investigation?
Hyperglycaemia = >7mmol/L
Acidosis pH <7.3
Ketones in urine
650
DKA management?
1) Resus ABC
2) Correct dehydration
3) Give insulin 1hr after giving fluids
4) Monitor - hourly blood glucose
Avoid bicarbonate as increases risk of cerebral oedema
651
Hypoglycaemia features?
- Irritable
- Sweaty
- Headache
- Pallor
- Drowsy
- Slurred speech
- Convulsions
652
HYPOTHYROIDISM
Congenital and acquired causes?
Congenital:
- iodine deficiency
- pituitary dysfunction
- maldescent of thyroid
Acquired:
- Prematurity
- Hashimoto's
- Trisomy 21
653
HYPOTHYROIDISM
Clinical features?
(Congenital normally picked up on screening and asymptomatic)
```
Bradycardia
Cold intolerance
Dry skin
Thin,dry hair
Constipation
Delayed puberty
Obesity
Learning difficulties
```
§
654
Treatment of Thyroid storm/crisis?
- IV hydrocortisone
- IV Fluids
- Propanolol
655
CONGENITAL ADRENAL HYPERPLASIA
How is it diagnosed?
Virilisation of external female genitalia
SALT-LOSERS:- (no aldosterone production)
- Low Na
- High K
- Metabolic acidosis
- Hypoglycaemia
656
Treatment of a salt-losing crisis?
Sodium chloride
Glucose
IV Hydrocortisone
657
What is Kallmann's?
Hypogonadotrophic hypogonadism causing:
- Delayed puberty
- Anosmia
658
Definition of Precocious puberty?
'development of secondary sexual characteristics before 8 years
in females and 9 years in males'
659
What is Thelarche?
First stage of breast development
660
What is Adrenarche?
First stage of pubic hair development
661
What is the Gonadotrophin pathway?
GnRH (from hypothalamus) —> ^FSH+LH —>
testes/ovaries —>
^testosterone & oestrogen/progesterone —> inhibits FSH/LH secretion
662
Ddx of pain with swelling?
- Trauma
- Infection (septic arthritis/osteomyelitis)
- JIA
- Arthritis (IBD)
- Vasculitis (HSP/Kawasaki's)
- CTD
- CF
- Sarcoidosis
- Developmental/congenital
663
JIA
Definiton?
Joint inflammation presenting in children and persisting for at least 6 weeks with other causes excluded
664
JIA
Classification?
Oligoarticular = 1-4 joints in first 6 months
Polyarticular RF -ve = 5+ joints in first 6 months
Polyarticular RF +ve = 5+ joints in first 6 months / +ve RF seen on two occasions
Systemic onset JIA = Arthritis + 2 weeks fever
Psoriatic = arthritis + psoriasis
665
JIA
Complications?
Chronic anterior uveitis
Growth failure
Osteoporosis
666
JIA
Investigations?
Normocytic anaemia
Raised: WCC, CRP, Platelets
RF / HLA-B27
MSK examination
X-rays
667
JIA
Treatment?
NSAIDs + Analgesics
Steroids
DMARDs (metho/sulfa)
Biologics
668
What is juvenile SLE?
Chronic autoimmune disease affecting every organ of the body
relapsing and remitting
669
Diagnostic criteria for Juvenile SLE?
SOAP BRAIN MD
Serositis (pleuritis/pericarditis)
Oral ulcers
Arthritis
Photosensitivity
```
Blood (pancytopenia)
Renal (proteinuria)
ANA +ve (anti-nuclear antibody)
Immunological
Neurological (psych/seizures)
```
Macular rash
Discoid rash
670
OSTEOMYELITIS
Where are the most common sites?
Distal femur
Proximal tibia
671
OSTEOMYELITIS
most common bacterial causes?
Staph Aureus
h.influenza
Group A beta haemolytic strep
672
OSTEOMYELITIS
Classic signs?
Severe pain
Immobile limb (pseudo paresis)
Swollen over area
Acute febrile illness (lethargy/high temp)
673
OSTEOMYELITIS
Investigations?
x-ray
MRI
blood cultures
FBC (^WCC/CRP)
674
OSTEOMYELITIS
Treatment?
(over 3 months old)
= IV Cefuroxime
SWITCH TO ORAL AFTER 1 WEEK IF CAN TOLERATE AND CRP <10:
Flucloxacillin if s.aureus
Co-amoxiclav if unsure
675
SEPTIC ARTHRITIS
What is it?
Serious infection of the joint space - can lead to bone destruction
676
What should you assume in a limping child?
Septic arthritis until proven otherwise
677
SEPTIC ARTHRITIS
Common organism causes?
1) S.aureus
| 2) h.influenza
678
SEPTIC ARTHRITIS
clinical presentation?
Red, hot, acutely tender joint
reduced ROM
Acutely febrile child
679
SEPTIC ARTHRITIS
Investigations?
Joint aspiration under USS = definitive
blood cultures
^WCC, CRP/ESR
680
SEPTIC ARTHRITIS
Treatment?
early treatment is vital to prevent destruction of articular cartilage + bone
IV Abx / possible surgical drainage
681
DDH
Risk factors?
- Female 6x more likely
- breech presentation
- Family Hx
- Firstborn child
- Oligohydramnios
682
DDH
Clinical presentation?
Screened in examination
BARLOW TEST = attempt to dislocate femoral head posteriorly
ORTOLANI TEST = attempt to relocate a dislocated femoral head
Observe for asymmetry
683
DDH
investigations?
Dynamic USS
early diagnosis important as if appropriately aligned in first few months = dysplastic hip can resolve spontaneously
684
DDH
Treatment?
Most will spontaneously stabilise in 3-6 weeks
Pavlik harness (flexion-abduction orthosis) in children under 4-5 months
685
PERTHE'S DISEASE
what is it?
How is it diagnosed?
Temporarily disrupted blood flow to femoral head causing avascular necrosis in children aged 4-8 years old
Limited abduction and internal rotation - abnormal ossification on x-ray
686
What is Achondroplasia?
Autosomal dominant condition that is the most common form of short-limb dwarfism due to REDUCED growth of cartilaginous bone
687
Clinical presentation of Achondroplasia?
- Large skull, normal trunk, short arms and legs
- Short stature
- Frontal bossing
- Marked lumbar lordosis
688
OSTEOPOROSIS
Definition?
Low bone mass and deterioration of bone tissue, leading to bone fragility and increased fracture risk
689
OSTEOPOROSIS
Possible causes?
Inherited - Osteogenesis Imperfecta
Acquired - Drug induced, malabsorption
690
OSTEOGENESIS IMPERFECTA
What is it?
Autosomal dominant condition causing increased fragility of bone
(collagen type 1 = bone/teeth)
691
OSTEOGENESIS IMPERFECTA
There is 4 types
Name some associated symptoms and treatment
Blue sclerae
Hearing loss
Bisphosphonates
692
Give examples of bisphosphonates?
Alendronate
Zoledronate
Pamidronate
693
ANAEMIA
What Hb figures show anaemia in
neonates?
1-12 months?
1-12 years?
o Neonate: Hb <140g/L
o 1month-12months: Hb<100g/L
o 1year-12years: Hb<110g/L
694
ANAEMIA
Causes?
- Impaired red cell production
(Iron deficiency,
- Increased red cell destruction (haemolytic disease of newborn, thalassaemias, sickle cell, G6PD deficiency)
- blood loss (meckel's diverticulum, von willebrand disease)
695
Signs of Anaemia?
jaundice -> kernicterus
Oedema
hepatosplenomegaly
696
Pathophysiology and treatment of haemolytic disease of a newborn?
1) negative rhesus mother and positive baby
2) baby has D antigen and mother does not
3) Mother produces antibody against D antigen and haemolysis of newborns RBCs
treatment?
give mother purified anti-D so she does not sensitise to babies antigen = not exposed to immune system = no haemolysis
697
Cause of iron-deficiency?
1) Inadequate intake (poor feeding)
2) Malabsorption (CMPI)
3) blood loss
698
Investigations of IDA?
- Microcytic hypo chromic reticulocytes
| - low ferritin and serum Fe
699
treatment of IDA?
Ferrous fumigate (oral iron therapy)
700
What is G6PD?
enzyme essential for preventing oxidative damage to red cells.
RBCs lacking G6PD = exposed to oxidant induced haemolysis
701
How is G6PD transmitted?
X-linked
702
G6PD
Presentation?
Neonatal jaundice
Acute intravascular haemolysis (triad signs below)
- Dark urine
- Fever
- Flank pain
703
What is sickle cell?
HbS forming due to mutation on chromosome 11.
the cells cannot flex through capillaries which leads to:
1) Blockage
2) Vessel occlusion
3) Ischaemia
4) Low O2 tension
(autosomal recessive)
704
How does HbS form?
Mutation on codon 6 of B-globulin gene - change in AA sequence from:
Glutamine to Valine
705
What is B-Thalassemia?
Excess of alpha chains
Autosomal recessive HBB gene on chromosome 11
HbA2 RAISED = ANAEMIA
706
Treatment of B-Thalassemia?
Long term folic acid
BM transplant
Regular transfusions
707
What is ITP?
Immune Thrombocyotpenic Purpura =
Destruction of platelets by IgG autoantibodies
708
Acute signs of ITP?
Petachie on extremities
Purpura/ bruising
709
Diagnosis?
Dx of exclusion, low platelets!
710
What is Von Willebrand Haemophilia?
Abnormality to vWF = no longer adhesive between platelets and damaged endothelium =
Excessive bleeding
711
Tx of vWF disorder?
Tranexamic acid (stop excessive bleeding post surgery)
712
ACUTE LYMPHOBLASTIC LEUKAEMIA (ALL)
What is it?
Disorder of lymphoid progenitor cells
Lymphoid precursors proliferate and replace normal cells of the bone marrow
713
What is the most common cancer in children?
Acute Lymphoblastic Leukaemia
714
Symptoms of ALL?
- Pancytopenia:
- Fatigue (anaemia)
- Increased infections (leukopenia)
- bleeding (thrombocytopenia)
- bone and joint pain
715
Cause of ALL?
abnormal chromosome number OR
chromosomal translocation of
[t(12;21] or
[t(9;22)] which is Philadelphia chromosome
716
Signs of ALL?
| what is seen on examination
- Hepatosplenomegaly (blast cells settle here so enlarge - abdominal distension)
- Lymphodenopathy (^ settle in lymph nodes)
- Petechiae
- Pallor
717
Diagnosis of ALL?
FBC - pancytopenia
Blood film - show blast cells
Bone marrow biopsy (CONFIRMS)
718
Treatment of ALL?
- Chemo/ Radio
| - BM/stem cell transplant
719
5 stages of Chemo?
1) Induction
2) Consolidation
3) Interim maintenance
4) Delayed intensification
5) Maintenance
720
Most common presentation of a Neuroblastoma?
Abdo mass (can be anywhere on sympathetic chain)
721
What is Wilm's tumour?
Most common renal tumour in childhood that is:
95% unilaterally
Presents as asymptomatic abdominal mass
722
Signs of raised ICP?
Headache
Change in behaviour
Nausea/vomiting
Over-sleepy
Papilloedema (may be decreased visual acuity)
723
Features of Retinoblastoma?
- Loss of red-reflex = replaced by white pupil (leukocoria)
724
Name the components of the traffic light system for an unwell child
1) Colour (skin, lips, tongue)
2) Activity
3) Respiratory
4) Circulation / Hydration
5) Other
725
Name high risk signs of an unwell child (Traffic light)
Colour :
Pallor, blue
Activity :
not responding to social cues, does not stay awake, high pitched cry
Respiratory :
grunting, tachypnoea, chest indrawing, RR >60
Circulation / Hydration :
reduced skin turgor
tachycardia
```
Other :
temperature
non-blanching rash
bulging fontanelle
neck stiffness
status epilepticus
focal signs/seizures
```
726
What heart rate is a high risk sign for the following ages:
<12 months
12-24 months
2-5 years
<12 months : >160 bpm
12-24 months : >150bpm
2-5 years : >140bpm
727
what temperature is a high risk sign for the following ages:
<3 months
3-6 months
<3 months : >38
3-6 months : >39
728
What breathing rate is a high risk sign for the following ages:
6-12 months
>12 months
6-12 months: >50
>12 months : >40
729
What is Respiratory Distress Syndrome?
Insufficient surfactant production seen in immature lungs
730
Risk factors of RDS?
Prematurity
| C-section
731
Clinical presentation of RDS?
```
Tachypnoea >60 min
Grunting
Nasal flaring
Intercostal recession
Cyanosis
```
732
Treatment of Respiratory Distress Syndrome?
give maternal corticosteroids (Betamethasone/dexomethasone)
= induce foetal lung maturation
Oxygen (CPAP)
exogenous surfactant by endotracheal tube
733
What is Bronchopulmonary Dysplasia?
Still needing O2 requirements after 36 weeks
| complication of RDS
734
What is MSAF?
meconium stained amniotic fluid.
This can lead to meconium aspiration syndrome when the baby inhales the meconium leading to Respiratory Distress
Treated with: Surfactant / inhaled nitric oxide
735
what is Chorioamnionitis?
Chorioamnionitis is a bacterial infection that occurs before or during labor. The name refers to the membranes surrounding the fetus: the “chorion” (outer membrane) and the “amnion” (fluid-filled sac). The condition occurs when bacteria infect the chorion, amnion, and amniotic fluid around the fetus
736
who is most likely to get Intussusception?
Male
| between 6-18 months
737
Diagnostic investigation for Intussusception?
Ultrasound
738
Treatment for Croup?
Single dose Dexamethsone/ prednisolone to reduce throat swelling
739
What is a Dermoid cyst?
Dermoid cysts are usually multiloculated and heterogeneous. Most are located above the hyoid
740
What are Cystic Hygromas?
Cystic hygromas are soft and transilluminate. Most are located in the posterior triangle.
741
Where will you find a Branchial cyst
smooth mass located on the lateral aspect of his anterior triangle, near to the angle of the mandible. On ultrasound; it has a fluid filled, anechoic, appearance
