Paediatrics Flashcards
1
Q
What is the ductus venosus?
A
Connects umbilical vein and IVC
Allows blood to bypass liver
2
Q
What is the role of foramen ovale?
A
Connects RA to LA
Allows blood to bypass RV and pulmonary circulation
3
Q
What is the role of the ductus arteriosus?
A
Connects pulmonary artery with aorta
Allows blood to bypass pulmonary circulation
4
Q
When does the foramen ovale close and what does it become?
A
Fossa ovalis
Closes at first breath- Breath expands alveoli, decreases pulmonary vascular resistance, causes fall in pressure in RA, LA pressure greater than RA = Squashes atrial septum and closes foramen ovale
5
Q
What can be given to keep the ductus arteriosus open?
A
Prostaglandin E
6
Q
Which shunt is affected by prostaglandin E?
A
Keeps ductus arteriosus open
7
Q
When does the ductus arteriosus close and what does it become?
A
Closes after 2-3d
Becomes ligamentum arteriosum
Increased blood oxygenation causes drop in circulating prostaglandins
8
Q
When does the ductus venosus close and what does it become?
A
Stops functioning after umbilical cord clamped
Structurally closes after 2-3wks
Becomes ligamentum venosum
9
Q
What are the features of innocent murmurs?
A
Soft
Short
Systolic
Symptomless
Situation dependent- Quieter on standing/only appears when unwell
10
Q
What features of a murmur would prompt further investigation?
A
Murmur louder than 2/6
Diastolic
Louder on standing
Failure to thrive/feeding difficulty/cyanosis/SOB
11
Q
What are the investigations done for murmurs?
A
ECG
Chest xray
ECHO
12
Q
Which murmurs are pansystolic?
A
Mitral regurgitation (mitral area)
Tricuspid regurgitation (tricuspid area)
Ventricular septal defect (left lower sternal border)
13
Q
List the ejection systolic murmurs
A
Aortic stenosis (aortic area)
Pulmonary stenosis (pulmonary area)
HCOM (4th ICS on L sternal border)
14
Q
What causes splitting of the 2nd heart sound?
A
Pulmonary valve closing slightly later than aortic valve
During inspiration chest wall and diaphragm pull lungs and heart open- Negative intrathoracic pressure- Causes R side of heart to fill faster as pulls blood in from venous system- Increased volume in RV means takes longer to empty causing delay in pulmonary valve closing
15
Q
Describe the examination findings of an atrial septal defect
A
Mid-systolic, crescendo-decrescendo loudest at upper left sternal border
Fixed split 2nd heart sound (blood flows from LA-RA increasing volume of blood in RV to empty before pulmonary valve can close)
16
Q
Describe examination findings of patent ductus arteriosus
A
Continuous crescendo-decrescendo machinery murmur that may continue during 2nd heart sound (makes 2nd heart sound difficult to hear)
17
Q
Which part of ToF dictates the severity of the condition?
A
The degree of pulmonary stenosis
18
Q
What does the murmur in ToF arise from?
A
Pulmonary stenosis- Ejection systolic murmur loudest in pulmonary area
19
Q
Which heart defects can causes cyanosis and why?
A
VSD
ASD
PDA
Transposition of great arteries
Cyanotic as cause R-L shunt
20
Q
What causes cyanosis in cyanotic heart disease?
A
Deoxygenated blood enters systemic circulation
Blood bypasses pulmonary circulation and lungs
Occurs across R-L shunt
21
Q
Which cyanotic heart defects are not always cyanotic and why?
A
VSD, ASD and PDA
Pressure in L heart>R heart, blood flows from high to low, prevents R-L shunt
If pulmonary pressure increases beyond systemic pressure = Starts R-L shunt and cyanosis = Eisenmenger syndrome
22
Q
Which cyanotic heart defect always causes cyanosis?
A
Transposition of Great Arteries
Right side of heart pumps directly into aorta and systemic circulation
23
Q
What is patent ductus arteriosus?
A
Fails to close (normally stops functioning within 1-3d and closes within 2-3wks)
Flow of blood from aorta to pulmonary artery
24
Q
What are the risk factors for developing a PDA?
A
Genetics
Maternal rubella infection
Prematurity
25
Outline presentation of PDA
Can be asymptomatic and close spontaneously
Can be asymptomatic throughout childhood and present in adulthood with signs of HF
SOB
Difficulty feeding
Poor weight gain
LRTIs
Continuous crescendo-decrescendo machinery murmur continues during 2nd heart sound
26
Outline pathophysiology of PDA
L-R shunt from aorta-pulmonary artery
Causes increase in pressure in pulmonary vessels= Pulmonary HTN, R heart strain, R ventricular hypertrophy
Increased blood through pulmonary vessels and returning to L side of heart = Left ventricular hypertrophy
27
Outline diagnosis of PDA
ECHO
Doppler flow studies
28
Outline management of PDA
Monitor until 1y with ECHO
Trans-catheter/surgical closure after 1y or sooner if symptomatic
29
Outline atrial septal defect
Hole in septum between 2 atria
L-R shunt
Can then lead to Eisenmenger syndrome
30
What is the difference between an atrial septal defect and foramen ovale?
Foramen ovale is normal until becomes patent after birth
Every atrial septal defect is abnormal
31
What are the complications of ASD?
*Stroke (VTE)- DVT travels to R side then crosses to left and can travel to brain
AF or atrial flutter
Pulmonary HTN and R sided HF
Eisenmenger syndrome
32
Outline presentation of ASD
Mid-systolic, crescendo-decrescendo murmur in upper L sternal border
Fixed split 2nd HS
SOB
Difficulty feeding
Poor weight gain
LRTIs
Adult- Dyspnoea, HF, stroke
Link between PFO and migraine with aura
33
Outline management of ASD
If small and asymptomatic- Watch and wait
Transvenous catheter closure or open heart surgery
Anticoagulants in adults- Aspirin/warfarin/NOACs
34
What is a VSD?
Congenital hole in septum between ventricles
35
Which genetic conditions are associated with VSD?
Downs syndrome
Turners syndrome
36
What is the direction of blood flow in VSD?
Increased pressure in LV means L to R meaning its acyanotic
Leads to right sided overload and right sided HF
Eventually switches to R to left shunt and Eisenmenger syndrome
37
Outline presentation of VSD
Initially asymptomatic and can present in adulthood
Poor feeding, dyspnoea, tachypnoea, failure to thrive
Pan systolic murmur in L lower sternal border in 3rd/4th ICS
Systolic thrill on palpation
38
What are the causes of a pansystolic murmur?
VSD
Mitral regurgitation
Tricuspid regurgitation
39
What is the management of VSD?
Small with no evidence pulmonary HTN or HF- Watch, can close spontaneously
Transvenous catheter closure via femoral vein or open heart surgery
40
What is there an increased risk of in VSD?
Infective endocarditis
41
What are the 3 underlying lesions that can result in Eisenmenger syndrome?
ASD
VSD
PDA
42
What is Eisenmenger Syndrome?
Blood flows from R to L across structural heart lesion, bypassing lungs
43
Which lesions can result in Eisenmenger syndrome?
ASD
VSD
PDA
44
What is the timeline for a lesion leading to Eisenmenger syndrome?
Develops after 1-2y in large shunts
Adulthood in small shunts
Develops quicker during pregnancy- If history of ‘hole in heart’ offer ECHO and close monitoring
45
List some findings related to R-L shunt and chronic hypoxia
Cyanosis
Clubbing
Dyspnoea
Plethora complexion (red complexion related to polycythaemia)
46
What is the cause of polupycythaemia?
Cyanosis
- Bone marrow responds to low oxygen sats and produces more RBCs and Hb to increase O2 carrying capacity of blood
Polycythaemia= Plethoric complexion due to high conc. Hb
47
What is a risk following polycythaemia?
Blood is more viscous due to higher conc. RBCs and O2= Increased risk thrombus formation
48
List examination findings of Eisenmenger syndrome
Right ventricular heave
Loud P2
Raised JVP
Peripheral oedema
ASD- Mid systolic, crescendo decrescendo murmur loudest at upper left sternal border
VSD- Pan systolic murmur loudest at lower left sternal border
PDA- Continuous crescendo decrescendo machinery murmur
49
What is the prognosis of Eisenmenger syndrome?
Can reduce life expectancy by approx. 20y
Causes of death- HF, VTE, infection, haemorrhage
Mortality can be up to 50% in pregnancy
50
What is the definitive management of Eisenmenger syndrome?
Correct underlying defect to prevent development of Eisenmenger syndrome
Once pulmonary pressure high enough and syndrome develops not reversible- Only treatment is heart-lung transplant
51
Outline medical management of Eisenmenger syndrome
Oxygen for symptom management
Sildenafil for pulmonary HTN
Treat arrhythmias
Venesection for polycythaemia
Anticoagulation for prevention thrombosis
Prophylactic ABs to prevent infective endocarditis
52
What is coarctation of the aorta?
Congenital condition
Narrowing of aortic arch, usually around ductus arteriosus
Reduces pressure of blood flowing to arteries distal to narrowing and increases pressure proximal to narrowing
53
Which genetic condition is associated with coarctation of aorta?
Turners syndrome
54
Outline presentation of coarctation of the aorta
Weak femoral pulses
Higher BP in limbs before narrowing and lower in limbs after narrowing
May have systolic murmur below left clavicle and left scapula
Tachypnoea and increased work of breathing
Underdeveloped left arm where reduced flow to left subclavian artery
Underdevelopment of legs
55
Outline management of coarctation of the aorta
Prostaglandin E to keep ductus arteriosus open whilst awaiting surgery
56
What is aortic valve stenosis?
Narrowing of aortic valve
Restricts blood flow from LV into aorta
57
Outline presentation of aortic valve stenosis
Can be asymptomatic
Fatigue
SOB
Dizziness
Fainting
Symptoms worse on exertion
Severe AS- Presents with HF within mths of birth
Ejection systolic murmur loudest in aortic area, crescendo-decrescendo radiating to carotids
Ejection click just before murmur, palpable thrill during systole, slow rising pulse and narrow pulse pressure
58
List complications of aortic stenosis
LV outflow tract obstruction
HF
Ventricular arrhythmias
Bacterial endocarditis
Sudden death, often on exertion
59
Outline diagnosis of aortic valve stenosis
ECHO
ECG
Exercise testing
60
Outline management of aortic stenosis
Percutaneous balloon aortic valvoplasty
Surgical aortic valvotomy
Valve replacement
61
What is comgenital pulmonary valve stenosis?
3 leaflets that can develop abnormally, becoming thickened or fused
Results in narrow opening between RV and pulmonary artery
62
List associations of pulmonary stenosis
ToF
William syndrome
Noonan syndrome
Congenital rubella syndrome
63
Outline presentation of pulmonary stenosis
Often asymptomatic
Fatigue on exertion
SOB
Dizziness
Fainting
Ejection systolic murmur loudest in pulmonary area
Palpable thrill in pulmonary area
RV heave due to RV hypertrophy
Raised JVP with giant a waves
64
Outline investigations of pulmonary stenosis
ECHO
65
Outline management of pulmonary stenosis
Balloon valvuloplasty via venous catheter
If valvuloplasty not appropriate- Open heart surgery
66
What are the 4 co-existing pathologies of Tetralogy of Fallot?
VSD
Overriding aorta
Pulmonary valve stenosis
Right ventricular hypertrophy
66
Which pathology in Tetralogy of Fallot determines severity?
Degree of pulmonary stenosis- Thickened valve means more deoxygenated blood encouraged to travel across VSD to travel into the aorta
67
What is an overriding aorta?
Entrance to aorta is further right than normal- When RV contracts and sends blood up, higher proportion of deoxygenated blood enters aorta
68
List risk factors for Tetralogy of Fallot
Rubella infection
Increased maternal age
Alcohol consumption in pregnancy
Diabetic mother
69
How is Tetralogy of Fallot investigated?
ECHO with doppler flow studies
Chest xray- Boot shaped heart due to RV hypertrophy
70
Outline presentation of Tetralogy of Fallot
Ejection systolic murmur caused by pulmonary stenosis
Severe- HF before 1y
71
List signs and symptoms of Tetralogy of Fallot
Cyanosis
Clubbing
Poor feeding
Poor weight gain
Ejection systolic murmur
Tet spells
72
What are Tet spells?
Intermittent symptomatic periods where R-L shunt becomes temporarily worsened precipitating a cyanotic episode
PVR increases or systemic resistance decreases
Precipitated by walking, physical exertion or crying
73
Outline non-pharmacological management of Tet spells
Older- Squat
Younger- Knees to chest
Both increases systemic vascular resistance, encouraging blood to enter pulmonary vessels
Supplementary oxygen
74
Outline pharmacological management of Tet spells
Beta blockers- Relaxes RV and improves flow to pulmonary vessels
IV fluids- Increases pre-load and volume of blood flowing to pulmonary vessels
Morphine- Can decrease respiratory drive= More effective breathing
Phenylephrine infusion- Increases SVR
75
Outline management of ToF
Prostaglandin infusion- Maintains ductus arteriosus
Total surgical repair- Open heart surgery
76
What is the prognosis of ToF?
Depends on severity
Poor without treatment
Corrective surgery= 90% patients live to adulthood
77
What is Ebstein's anomaly?
Tricuspid valve set low in right side of heart (towards apex)
Causes bigger RA and smaller RV
Leads to poor flow to pulmonary vessels
Often associated with R-L shunt across atria via ASD
Cyanosis
78
Which syndrome is Ebtein's anomaly associated with?
Wolff-Parkinson-White Syndrome
79
Outline presentation of Ebstein's anomaly
HF- Oedema
Gallop rhythm with 3rd and 4th HS
Cyanosis
SOB and tachycardia
Poor feeding
Collapse or cardiac arrest
80
When does Ebstein's anomaly present?
A few days after birth when ductus arteriosus closes
81
How is Ebstein's anomaly diagnosed?
ECHO
82
Outline management of Ebstein's anomaly
Treat arrhythmias and HF
Prophylactic ABs to prevent infective endocarditis
Surgical correction
83
84
85
86
Which conditions are associated with Transposition of the GReat arteries?
VSD
Coarctation of the aorta
Pulmonary stenosis
86
What is the pathophysiology of Transposition of the Great Arteries?
Aorta and pulmonary trunk swapped
RV pumps blood into aorta and LV pumps blood into pulmonary vessels
Immediately life threatening
CYANOSED
86
Outline presentation of Transposition of Great Arteries
Cyanosis at or within a few days of birth
Patent ductus arteriosus can initially compensate
Respiratory distress
tachycardia
Poor feeding
Poor weight gain
Sweating
87
Outline management of Transposition of Great Arteries
VSD allows for mixing of blood to provide time for definitive treatment
Prostaglandin infusion- Maintains ductus arteriosus
Balloon septostomy- Insert catheter in foramen ovale via umbilicus- Inflate balloon to create large ASD
Open heart surgery definitive- Cardiopulmonary bypass
88
What is bronchiolitis?
Inflammation and infection in bronchioles
Common in winter
Children <1y, most common <6mths
89
What is the causative organism of bronchiolitis?
Respiratory syncytial virus (RSV)
90
Outline presentation of bronchiolitis
Coryzal- Snotty, sneezy, mucus in throat, watery eyes
Signs of resp. distress
Dyspnoea
Tachypnoea
Poor feeding
Mild fever (<39 degrees C)
Apnoeas
Wheeze and crackles on auscultation
91
What are the signs of respiratory distress?
Raised RR
Use of accessory muscles
Intercostal and subcostal recessions
Nasal flaring
Head bobbing
Tracheal tugging
Cyanosis
Abnormal airway noises
92
What is the function of grunting?
Caused by exhaling with glottis partially closed- Increases PEEP
93
What is stridor?
High pitched inspiratory noise caused by obstruction of upper airway
94
What is the typical course of RSV?
Bronchiolitis:
Starts as URTI with coryzal symptoms
Chest symptoms over 1st 2 days following onset coryzal symptoms
Symptoms generally worst on day 3-4
Symptoms last 7-10d
Most fully recover within 2-3wks
Increases incidence of viral induced wheeze later in childhood
95
When shoudl you consider admission in children with bronchiolitis?
<3mths or any pre-existing conditions (prematurity, Down syndrome, CF)
<75% normal milk intake
Clinical dehydration
RR >70
O2 sats <92%
Respiratory distress
Apnoeas
Parents not confident in managing condition at home
96
Outline management of bronchiolitis
Supportive management:
Ensure adequate intake
Saline nasal drops and suctioning
Supplementary oxygen
Ventilatory support:
High-flow humidified oxygen
CPAP
Intubation and ventilation
97
What are the most helpful signs of poor ventilation on ABG?
Rising pCO2- Shows airways collapsed, can't clear waste CO2
Falling pH- CO2 is building up
Respiratory acidosis
If also hypoxic- Type 2 respiratory failure
98
What is the role of Palivizumab in bronchiolitis?
Targets RSV
Give as prophylaxis mthly
Given to high risk- Ex-premature or CHD
Provides passive protection
99
What is a viral induced wheeze?
Acute wheezy illness caused by viral infection
Small children have small airways- Small inflammation restricts small airways more and constricts SM
100
Which law is associated with viral induced wheeze?
Poiseuille's law
Flow rate is proportional to radius of tube to power of 4
Eg: Halving diameter of tube decreases flow rate by 16 fold
101
Outline management of viral induced wheeze
Same pathway as acute asthma in children
102
Outline presentation of viral induced wheeze
Evidence of viral illness for 1-2d preceding onset of:
SOB
Signs of respiratory distress
Expiratory wheeze throughout chest
103
What are the causes of a focal wheeze?
Focal airway obstruction:
Inhaled foreign body
Tumour
104
What are the features of viral induced wheeze compared to asthma?
Presents before 3y age
No atopic history
Only occurs during viral infections
105
What are the potential triggers of asthma?
Infection
Exercise
Cold weather
Animals
Dust
Smoke
Doof allergens
106
Outline presentation of asthma
Progressively worsening SOB
Signs of resp. distress
Tachypnoea
Expiratory wheeze throughout chest
Chest can sound 'tight' on auscultation, with reduced air entry
Silent chest- Life-threatening
107
Outline moderate severity of acute asthma
Peak flow >50% predicted
Normal speech
108
Outline severe presentation of acute asthma
Peak flow <50% predicted
Sats <92%
Unable to complete sentences in one breath
Signs of resp. distress
Resp rate: >40 1-5y, >30 >5y
HR: >140 1-5y, >125 >5y
109
Outline life-threatening presentation in acute asthma
Peak flow <33% predicted
Sats <92%
Exhaustion and poor resp effort
Hypotension
Silent chest
Cyanosis
Altered consciousness/confusion
110
Outline management of severe acute asthma
Supplemetary O2
Inhaled/nebulised salbutamol
Inhaled/nebulised ipratropium bromide
IV magnesium sulphate
IV aminophylline
Also give oral prednisolone (for 3d) or IV hydrocortisone
111
Outline management of mild acute asthma
Outpatient regular salbutamol inhaler via spacer (eg: 4-6 puffs every 4h)
112
Outline step down of acute asthma management
Work back down ladder
Must have been off nebuliser at least 24h before discharged
Typical stepdown regime: Inhaled salbutamol 10 puffs 2hrly, 10 puffs 4hrly, 6 puffs 4hrly, 4 puffs 6hrly
Monitor potassium
113
What are the SEs of salbutamol?
Tachycardia
Tremor
Hyperkalaemia
114
When can a patient be discharged following an acute asthma attack?
Considered well- 6 puffs 4hrly salbutamol
Prescribe reducing regime for at home
Finish course of steroids
115
What is asthma?
Chronic inflammatory airway disease
Smooth muscle of airway hypersensitive, responds to stimuli by constricting and causing airflow obstruction
Bronchoconstriction reversible with bronchodilators (salbutamol)
116
What are the atopic conditions?
Asthma
Eczema
Hay fever
Food allergies
117
Outline presentation suggesting diagnosis of asthma
Episodic symptoms with intermittent exacerbations
Diurnal variability (worse at night and early morning)
Dry cough with wheeze and SOB
Typical triggers
Atopic conditions
FHx Asthma or atopy
Bilateral widespread 'polyphonic wheeze'
Symptoms improve with bronchodilators
118
Outline diagnosis of asthma
Spirometry with reversibility testing
Direct bronchial challenge test (with histamine or methacholine)
FeNO
Peak flow variability (diary)
119
Outline long term management of asthma in <5y
1. SABA inhaler (salbutamol)
2. Low dose ICS or LTRA (montelukast)
3. Add other option from step 2
4. Refer
120
Outline medical long term management of asthma in 5-12y
1. SABA
2. Low dose ICS
3. LABA (salmeterol)- Only continue if good response
4. Medium dose ICS- COnsider adding LTRA (montelukast) or oral theophylline
5. High dose ICS
6. Refer
121
Outline long term asthma management >12y
1. SABA
2. Low dose ICS
3. LABA (salmeterol)- Only continue if good response)
4. Medium dose ICS- Consider LTRA or oral theophylline or LAMA (tiotropium)
5. High dose ICS and combine step 4 with option of oral salbutamol and refer
122
Outline ICS use in children
Can slightly reduce growth velocity and cause small reduction in final height if used >12mths
Less of a problem with low doses
Effect of poorly controlled asthma is worse
123
Outline presentation of pneumonia
Cough (wet and productive)
High fever
Tachypnoea
Tachycardia
Increased WOB
Lethargy
Delirium
Hypoxia
Hypotension
124
What are the characteristic chest signs of pneumonia?
Bronchial breath sounds- Harsh breath sounds, equally loud on inspiration and expiration, caused by consolidation of lung tissue around airway
Focal coarse crackles- Air passing through sputum
Dullness to percussion- Lung collapse and/or consolidation
125
What is the most common cause of pneumonia?
Strep pneumonia
126
When is Group B strep pneumonia common?
Pre-vaccinated infants
Often contracted at birth as colonises vagina
127
What are typical findings of staph aureus pneumonia?
CXR- Pneumatocoeles (round air filled cavities) and consolidation in multiple lobes
128
When is haemophilus influenza a common cause of pneumonia?
Pre-vaccinated/unvaccinated
129
When is mycoplasma pneumonia considered as the cause of the pneumonia?
Atypical bacteria with extra-pulmonary manifestations (eg: Erythema multiforme)
130
What is the most common cause of viral pneumonia?
RSV
131
Outline investigations of pneumonia
CXR
Sputum cultures and throat swabs for bacterial cultures and viral PCR
Blood cultures
Capillary blood gas
132
Outline management of pneumonia
1st line- Amoxicillin
Add macrolide (erythromycin/clarithromycin/azithromycin) to cover atypical
IV if sepsis or problem with intestinal absorption
Oxygen as required
133
Outline management of recurrent LRTIs
FBC
CXR
Serum Igs
Test IgG to previous vaccines- Immunoglobulin class-switch recombination deficiency (unable to convert IgM to IgG)
Sweat test- CF
HIV test
134
What is croup?
Acute infective respiratory disease
6mth-2y
URTI causing oedema in larynx
Caused by parainfluenza virus
135
What is the most common causative organism of croup?
Parainfluenza virus
136
Why is diphtheria vaccinated against?
Causes croup
Croup caused by diphtheria leads to epiglottitis and has high mortality
137
Outline presentation of croup
Increased WOB
Barking cough
Hoarse voice
Stridor
Low grade fever
138
Outline management of croup
Oral dexamethasone- Very effective
Severe:
1. Oral dex
2. Oxygen
3. Nebulised budesonide
4. Nebulised adrenalin
5. Intubation and ventilation
139
What is the most common cause of epiglottitis?
Haemophilus influenza type B (HiB)- Rare now vaccinated against
140
Outline presentation suggesting possible epiglottitis
Sore throat and stridor
Drooling
Tripod position
High fever
Difficulty or painful swallowing
Muffled voice
Scared and quiet child
Septic and unwell appearance
141
Outline investigations of epiglottitis
DO NOT EXAMINE
Lateral xray of neck- Thumbprint sign (shouldn't really xray)
142
Outline management of epiglottis
DO NOT EXAMINE PATIENT
Alert most senior paediatrician and anaesthetist
Prepare for an intubation (may not be required but need to be ready at all times)
Once airway secure:
IV antibiotics- Ceftriaxone
Dexamethasone
143
Outline prognosis of epiglottis
Most recover w/o requiring intubation
If intubated, most extubated after a few days and make full recovery
144
What is a common complication of epiglottitis?
Epiglottic abscess- Collection of pus around epiglottitis
LIFE THREATENING EMERGENCY
145
What is laryngomalacia?
Part of larynx above vocal cords causes partial airway obstruction
Leads to chronic stridor on inhalation when larynx flops across airway
Usually resolves as larynx matures
146
What is the classic shape of the epiglottis in laryngomalacia?
Omega shape
147
Outline presentation of laryngomalacia
Infants, peaks at 6mths
Inspiratory stridor- Intermittent and more prominent when feeding/upset/lying on back
No associated respiratory distress
Can cause difficulty feeding
148
Outline whooping cough
URTI
Pertussis (bacteria)
Coughing fits so severe, unable to take in air between coughs- Whoop to forcefully suck air in
Vaccinated
NOTIFIABLE
149
Outline presentation of whooping cough
Start: Mild coryzal symptoms, low grade fever and mild dry cough
More severe coughing after a week- Sudden and recurring attacks of coughing with cough free periods between- Paroxysmal
Large inspiratory whoop
Can cough so hard they give themselves a pneumothorax
150
Outline diagnosis of whooping cough
Nasopharyngeal/nasal swab with PCR testing or bacterial culture- Can confirm diagnosis within 2-3wks onset of symptoms
If cough present >2wks- Can test for anti-pertussis toxin IgG
151
Outline management of pertussis
Supportive care
Within 21d- Macrolides (azithromycin, erythromycin, clarithromycin)
Co-trimoxazole alternative
Close contacts require prophylactic antibiotics if in vulnerable group
152
What is a key complication of whooping cough?
Bronchiectasis
153
What is CLDP?
Chronic lung disease of prematurity- Bronchopulmonary dysplasia
Those born <28wks gestation- Suffer with RDS and require oxygen therapy/intubation and ventilation at birth
154
How is CLDP diagnosed?
Based on CXR changes and when infant requires O2 therapy after reach 36wks
155
Outline feature of CLDP
Low O2 sats
Increased WOB
Poor feeding and weight gain
Crackles and wheeze
Increased susceptibility to infection
156
Outline prevention methods of CLDP before infants birth
Betamethasone (corticosteroids) to mother's showing signs of premature labour at <36wks gestation- Speeds up development of fetal lungs before birth
157
Outline prevention methods of CLDP in newborn
CPAP rather than intubation and ventilation when possible
Use caffeine to stimulate respiratory effort
Do not over oxygenate
158
Outline management of CLDP
Protect against RSV- Reduce risk and severity of bronchiolitis
Give mthly injections palivizumab
159
Outline CF
Autosomal recessive
Affects mucus glands
Mutation of CFTCR gene on Chr7
160
What are the key consequences of the cystic fibrosis mutation?
Thick pancreatic and biliary secretions- Block ducts- Lack of digestive enzymes
Low volume thick airway secretions- Reduce airway clearance- Bacterial colonisation and infection susceptibility
Congenital bilateral absence of vas deferens
161
Outline presentation of CF
Newborn spot test
Meconium ileus- First sign of CF- Meconium thick and sticky- Gets stuck and obstructs bowel- Not passing meconium within 24h birth, abdo distension and vomiting
162
How can CF present later in childhood?
Recurrent LRTIs
Pancreatitis
Failure to thrive
163
List symptoms of CF
Chronic cough
Thick sputum production
Recurrent RTIs
Loose, greasy stools (steatorrhoea)- Lack of fat digesting lipase enzymes
Abdo pain and bloating
Salty sweat
Failure to thrive
164
List signs of CF
Low weight or height on growth charts
Nasal polyps
Finger clubbing
Crackles and wheezes on auscultation
Abdo distension
165
List causes of clubbing in children
Hereditary clubbing
Cyanotic HD
Infective endocarditis
CF
TB
IBD
Liver cirrhosis
166
Outline diagnosis of CF
Newborn blood spot testing
Sweat test- Gold standard
Genetic testing- CFTR (amniocentesis or CVS)
167
Outline the sweat test
Used to diagnose CF
Pilocarpine applied to skin on patch
Electrodes placed either side causing skin to sweat
Chloride conc.- CF >60mmol/l
168
What are the key colonisers in CF?
Staph aureus
Pseudomonas aeruginosa
169
Outline CF and Pseudomonas
Once colonised with pseudomonas- Very difficult to get rid of
Treatment- Long term nebulised ABs (tobramycin) and oral ciprofloxacin
170
Outline management of CF
Chest physiotherapy- Clear mucus, reduce risk of infection
Exercise
High calorie diet
CREON tablets- Digest fats in pancreatic insufficiency
Prophylactic flucloxacillin
Salbutamol as required
Nebulised DNase- Breaks down DNA material in respiratory secretions
Nebulised hypertonic saline
Vaccines- Pneumococcal, influenza, varicella
171
Outline monitoring requirements in CF
Clinic every 6mths
Monitor sputum for colonisation
Screening- Diabetes, osteoporosis, Vit D deficiency, liver failure
172
Outline prognosis of CF
Life expectancy approx. 47y
90% develop pancreatic insufficiency
50% develop CF-related diabetes and require insulin
30% develop liver disease
173
What is primary ciliary dyskinesia?
Kartagner's syndrome
Autosomal recessive
Affects cilia of various cells in the body
More common in consanguineous communities (parents related)
Build up of mucus in lungs- Similar presentation to CF
Affects cilia in fallopian tubes and flagella of sperm- Reduced fertility
174
Which condition is primary ciliary dyskinesia associated with?
Situs inversus
175
What is Kartagner's triad?
Paranasal sinusitis
Bronchiectasis
Situs inversus
176
Outline diagnosis of primary ciliary dyskinesia
FHx and history of consanguinity in parents
CXR- Situs inversus
Semen analysis
Sample of ciliated epithelium- Nasal brushing or bronchoscopy
177
Outline management of PCD
Similar management to CF and bronchiectasis
178
What are the complications of appendicitis?
Gangrene and rupture leading to peritonitis
179
When is the peak incidence of appendicitis?
10-20y
180
What are the signs and symptoms of appendicitis?
Abdo pain- Central moving to RIF
Tenderness in McBurney's point
Loss of appetite
Nausea and vomiting
Rovsing's sign- Palpate LIF causes pain in RIF
Abo guarding and rebound and percussion tenderness- Suggests peritonitis
181
What are the signs of peritonitis in appendicitis?
Rebound tenderness and percussion tenderness caused by ruptured appendix
182
Outline diagnosis of appendicitis
Clinical presentation and raised inflammatory markers
CT scan
US- Rule out gynae causes
Diagnostic laparoscopy- Diagnose and perform appendicectomy in same procedure
183
Outline management of appendicitis
Laparoscopic surgery- Appendicectomy
184
What is Meckel's diverticulum?
Malformation of distal ileum
Usually asymptomatic
Can bleed/inflame/rupture causing volvulus or intussusception
185
What is mesenteric adenitis?
Inflamed abdominal lymph nodes
Abdo pain
Usually in younger children
Associated with tonsillitis or URTI
No treatment required
186
What is intussusception?
Bowel telescopes into itself
Thickens size of bowel and narrows lumen
Palpable mass and obstruction to passage of faeces
More common in 6mth-2y boys
187
Which conditions are associated with intussusception?
Concurrent viral illness
Henoch-Schonlein purpura (HSP)
CF
Intestinal polyps
Meckel diverticulum
188
Outline presentation of intussusception
Redcurrent jelly stool
Colicky abdomen
PAle, lethargic, unwell
RUQ sausage-shaped mass
Vomiting
Intestinal obstruction
189
Outline management of intussusception
US or contrast enema
Therapeutic enema- Air/water/contrast pumped into colon to try to force bowel into normal position
Surgical reduction
If gangrenous/perforated- Surgical resection
190
What are the complications of intussusception?
Obstruction
Gangrenous bowel
Perforation
Death
191
What is Hirschsprung's disease?
Congenital
Nerve cells of myenteric plexus absent in distal bowel and rectum
Bowel loses motility
192
What is total colonic aganglionosis?
Hirschsprung's disease
Aganglionic section of colon doesn't relax, causing it to become constricted- Loss of movement of faeces and obstruction
193
What are the associations with Hirschsprung's disease?
Downs syndrome
NF
Waardenburg syndrome
Multiple endocrine neoplasia type II
194
Outline presentation of Hirschsprung's disease
Delay in passing meconium >24h
Chronic constipation since birth
Abdo pain and distension
Vomiting
Poor weight gain and failure to thrive
195
What is Hirschsprung-associated enterocolitis?
Inflammation and obstruction of intestine
Presents within 2-4wks of birth
Fever, abdo distension, diarrhoea, features of sepsis
LIFE THREATENING
196
What are the complications of Hirschsprung-associated enterocolitis?
Toxic megacolon and perforation of bowel
Requires urgent ABs, fluid resus, decompression of obstructed bowel
197
Outline management of Hirschsprung's disease
Abdo xray
Rectal biopsy- Absence of ganglionic cells
If enterocolitis- Initial fluid resus and management of intestinal obstruction- IV ABs
Surgical removal of aganglionic section of bowel
198
List causes of intestinal obstruction
Meconium ileus
Hirschsprung's disease
Oesophageal atresia
Intussusception
Imperforate anus
Malrotation of intestines with volvulus
Strangulated hernia
199
Outline presentation of intestinal obstruction
Persistent vomiting- May be bilious
Abdo pain and distension
Failure to pass stools/wind
Abnormal bowel sounds- High pitched and tinkling early, absent later
200
Outline diagnosis of intestinal obstruction
Abdo xray- Dilated loops of bowel proximal, collapsed loops distal
Absence of air in rectum
201
Outline management of intestinal obstruction
Nil by mouth
Nasogastric tube to drain stomach and stop vomiting
IV fluids to correct dehydration
Paediatric surgical referral
202
What is biliary atresia?
Congenital
Section of bile duct narrowed or absent
Cholestasis- Bile cannot be transported from liver to bowel- Conjugated bile not excreted
203
Outline presentation of biliary atresia
Presents shortly after birth- Sig. persistent jaundice due to high conjugated bilirubin levels (Lasts >14d)
204
How is biliary atresia diagnosed?
Conjugated and unconjugated bilirubin
High conjugated bilirubin- Liver processing bilirubin for excretion but not able to flow through biliary duct into bowel
205
Outline management of biliary atresia
Kasai portoenterostomy
Often require full liver transplant
206
What are the features of Crohn's?
N- No blood or mucus
E- Entire GI tract
S- Skip lesions on endoscopy
T- Terminal ileum most affected and Transmural (full thickness) inflammation
S- Smoking RF
Associated with weight loss/strictures/fistulas
Diarrhoea, abdo pain, bleeding, weight loss, anaemia
207
What are the features of Ulcerative Colitis?
C- Continuous inflammation
L- Limited to colon and rectum
O- Only superficial mucosa affected
S- Smoking protective
E- Excrete blood and mucus
U- Use aminosalicylates
P- Primary sclerosing cholangitis
Diarrhoea, abdo pain, bleeding, weight loss, anaemia
208
What are the extra-intestinal manifestations of IBD?
Finger clubbing
Erythema nodosum
Pyoderma gangrenosum
Episcleritis and iritis
Inflammatory arthritis
Primary sclerosing cholangitis (UC)
209
How is IBD diagnosed?
Faecal calprotectin
Endoscopy- Gold standard
Imaging- US, CT, MRI- Look for complications (fistulas, abscesses, strictures)
Bloods- Infection, anaemia, thyroid, kidney, liver function
210
Outline inducing remission in Crohn's
1st line- Steroids (eg: Oral prednisolone or IV hydrocortisone)
2nd line- Immunosuppression- Azathioprine/mercaptopurine/methotrexate/infliximab/adalimumab
211
Outline maintaining remission in Crohn's
1st line- Azathioprine, mercaptopurine
2nd line- Methotrexate, infliximab, adalimumab
212
Outline inducing remission in UC
Mild to moderate:
1st line- Aminosalicylate (eg: Mesalaxine)
2nd line- Corticosteroids (eg: Prednisolone)
Severe:
1st line- IV corticosteroids (eg: Hydrocortisone)
2nd line- IV ciclosporin
Can surgery- Remove colon and rectum to form J pouch
213
Outline maintaining remission in UC
Aminosalicylate (eg: Mesalazine)
Azathioprine
Mercaptopurine
214
Which antibodies are associated with coeliac disease?
Anti-TTG
Anti-EMA
215
What is coeliac disease?
AI
Exposure to gluten causes immune reaction- Creates inflammation in small intestines
Particularly jejunum
Causes atrophy of intestinal villi
216
Outline presentation of coeliac disease
Failure to thrive
Diarrhoea
Fatigue
Weight loss
Mouth ulcers
Anaemia secondary to iron, B12 or folate deficiency
Dermatitis herpetiformis
Rarely neuro- Peripheral neuropathy, cerebellar ataxia, epilepsy
217
Which condition is dermatitis herpetiformis associated with?
Coeliac disease
218
What is the most common gene association of coeliac disease?
HLA-DQ2
219
What condition is always tested for in patients newly diagnosed with T1D?
Coeliac disease
220
What is the link between IgA deficiency and coeliac disease?
Anti-TTG and anti-EMA are IgA
When testing for these antibodies-Test total IgA levels- If total IgA is low- Coeliac test will be negative even if have condition
221
Outline diagnosis of coeliac disease
Must be on diet containing gluten to test
Check Total IgA levels to exclude IgA deficiency
Antibodies- Anti-TTG (1st choice) and Anti-EMA
Endoscopy and intestinal biopsy- Crypt hypertrophy, villous atrophy
222
What is seen on an endoscopy and intestinal biopsy in coeliac disease?
Crypt hypertrophy
Villous atrophy
223
List associations with coeliac disease
T1D
Thyroid disease
AI hepatitis
PBC
PSC
Down syndrome
224
What are the complications of untreated coeliac disease?
Vit deficiency
Anaemia
Osteoporosis
Ulcerative jejunitis
Enteropathy-associated T-cell lymphoma (EATL) of intestine
NHL
Small bowel adenocarcinoma
225
Outline management of coeliac disease
Lifelong gluten free diet
226
What us acute gastritis?
Inflammation of stomach- Presents with nausea and vomiting
227
What is enteritis?
Inflammation of intestines
Presents with diarrhoea
228
Outline management of gastroenteritis?
Isolate patient
Treat dehydration
ABs generally not recommended
229
What are the most common causes of viral gastroenteritis?
Rotavirus
Norovirus
Adenovirus (less common- More subacute diarrhoea)
230
Outline E.coli gastroenteritis
Only certain strains cause gastroenteritis
Spread contact with faeces/unwashed salad/contaminated water
Produces shiga toxin- Leads to HUS- Use of ABs increases risk
Abdo cramps, bloody diarrhoea, vomiting
231
Outline Campylobacter Jejuni as a cause of gastroenteritis
Traveller's diarrhoea
Gram negative spiral bacteria
Spread- Raw poultry, untreated water, unpasteurised milk
Incubation 2-5d, symptoms resolve after 3-6d
Abdo cramps, diarrhoea with blood, vomiting, fever
Azithromycin or ciprofloxacin- If severe
232
Outline Shigella as a cause of gastroenteritis
Spread- Faeces contaminating drinking water/swimming pools/food
Incubation 1-2d, resolve within 1wk
Bloody diarrhoea, abdo cramps, fever
Can produce shiga toxin and cause HUS
Treat severe cases- Azithromycin or ciprofloxacin
233
Outline salmonella as a cause of gastroenteritis
Spread- Raw eggs/poultry, food contaminated with infected faeces of small animals
Incubation 12h-3d, symptoms resolve within 1wk
Watery diarrhoea with mucus/blood, abdo pain, vomiting
Antibiotics only if severe- Guided by stool culture and sensitivities
234
Outline Bacillus Cereus as a cause of gastroenteritis
Gram positive rod
Rice left at room temperature
Growing on food- Produces cereulide toxin- Causes abdo pain, cramping, vomiting within 5h- In intestine produces different toxins that cause watery diarrhoea within 8h- Resolves within 24h
235
Outline Yersinia Enterocolitica as a cause of gastroenteritis
Gram negative bacillus
Eating raw/undercooked pork, spread through contamination with urine/faeces of rats and rabbits
Children, water/bloody diarrhoea. abdo pain, fever and lymphadenopathy
Can last >3wks
Older children- Mesenteric lymphadenitis causing R sided abdo pain
236
Outline Staph aureus toxin as a cause of gastroenteritis
Can grow on eggs/dairy/meat
Diarrhoea, perfuse vomiting, abdo cramps, fever
Start within hrs of digestion and settle within 12-24h
237
Outline Giardiasis as a cause of gastroenteritis
Parasite
Spread via pets, farmyard animals and humans
Faecal oral transmission
May cause diarrhoea or no symptoms
Diagnose- Stool microscopy
Treat- Metronidazole
238
Outline principles of gastroenteritis management
Infection control
Stay off school until 48h after symptoms completely resolved
Stool MC&S
Attempt fluid challenge
Rehydration solution- Dioralyte
Antidiarrhoeal meds not recommended
239
Outline post-gastroenteritis complications
Lactose intolerance
IBS
Reactive arthritis
Guillain-Barre syndrome
240
Outline pyloric stenosis
Hypertrophy of pyloric sphincter
Increasingly powerful peristalsis in stomach results in projectile vomiting
241
Outline features of pyloric stenosis
Presents within 1st few wks of life
Hungry baby, thin, failure to thrive
Projectile vomiting
Olive shaped mass in upper abdomen
Blood gas- Hypochloric metabolic alkalosis
242
What would a blood gas show in pyloric stenosis?
Hypochloric metabolic alkalosis
243
Outline management of pyloric stenosis
Diagnose- Abdo US
Treat- Laparoscopic pyloromyotomy (Ramstedt's operation)
Prognosis- Excellent
244
What is Gastro-oesophageal reflux?
Contents of stomach reflux through lower oesophageal sphincter
In babies- Immaturity of LOS- Usually resolves by 1y
245
Outline presentation of GOR
Chronic cough
Hoarse cry
Distress, crying, unsettled after feeding
Reluctance to feed
Pneumonia
Poor weight gain
>1y- Heartburn, acid regurg, retrosternal/epigastric pain, bloating, nocturnal cough
246
Outline management of GOR
Small, frequent meals
Burping regularly
Not over-feeding
Keep baby upright after feeding (i.e. not lying flat)
More problematic cases:
Gaviscon mixed with feeds
Thickened milk/formula
PPIs (omeprazole)
Rarely may require barium meal and endoscopy
Surgical fundoplication if very severe- Rare
247
What is Sandifer's syndrome?
Rare condition
Brief episodes of abnormal movement associated with GOR
Usually neurologically normal
Torticollis- Forceful contraction of neck muscles causing twisting of neck
Dystonia- Abnormal contractions causing twisting movements, arching back/unusual postures
Resolves as reflux improves
REFER as differential diagnoses- Infantile spasms and seizures
248
Outline presentation of constipation
<3 stools/wk
Hard stools, difficult to pass
Rabbit dropping stools
Straining/painful passage of stool
Abdo pain
Retentive posturing
Rectal bleeding associated with hard stools
Faecal impaction causing overflow soiling
Hard stools may be palpable in abdo
Loss of sensation of need to open bowels
249
What is encopresis?
Faecal incontinence
Not pathological until 4y
Usually sign of chronic constipation- Rectum stretched and loses sensation
250
What is desensitisation of the rectum?
Patients develop habit of not opening bowels when need to- Ignore sensation of full rectum
Lose sensation over time and open bowels less frequently
Leads to faecal impaction
Makes more difficult to treat constipation
251
What are the secondary causes of constipation?
Hirschsprung's disease
CF
Hypothyroidism
SC lesions
Sexual abuse
Intestinal obstruction
Anal stenosis
Cows milk intolerance
252
What are the complications of constipation?
Pain
Reduced sensation
Anal fissures
Haemorrhoids
Overflow soiling
Psychosocial morbidity
253
Outline management of chronic constipation
High fibre diet, good hydration
Movicol 1st line laxative
Disimpaction regime if faecal impaction
Encourage and praise visiting toilet
254
Outline abdominal migraines
May occur before develop traditional migraines when older
Episodes of central abdo pain lasting >1h
255
What are the features of abdominal migraine?
Central abdo pain lasting >1h
Nausea and vomiting
Anorexia
Pallor
Headache
Photophobia
Aura
256
Outline acute management of abdominal migraine
Low stimulus environment (quiet, dark room)
Paracetamol
Ibuprofen
Sumatriptan
257
Outline preventative management of abdominal migraine
Pizotifen
258
What are the withdrawal SEs of pizotifen?
Depression
Anxiety
Poor sleep
Tremor
259
What is T1d?
Pancreas can't produce insulin
Glucose can't get into cells- Cells think no glucose in body- Hyperglycaemia
260
What is the ideal blood glucose conc.?
4.4-6.1mmol/l
261
Outline production and role of insulin
Produced by pancreas by beta cells in Islets of Langerhans
Reduces blood sugar levels
Causes cells to absorb glucose
Causes muscle and liver cells to absorb glucose and store as glycogen
262
Outline production and use of glucagon
Increases blood sugar levels
Produced by alpha cells in Islets of Langerhans in pancreas
Stimulates liver to breakdown glycogen into glucose (glycogenolysis) and convert proteins and fats into glucose (gluconeogenesis)
263
Outline ketogenesis
Occurs when insufficient supply of glucose and glycogen stores depleted
Liver converts fatty acids into ketones
Water soluble fatty acids- Can cross BBB and be used by the brain
Acetone smell if in ketosis
264
Outline presentation of T1D
Often present in DKA
or
Hyperglycaemia:
Polyuria
Polydipsia
Weight loss
Secondary enuresis and recurrent infections
265
How is T1D diagnosed?
Bloods- FBC, renal profile, formal lab glucose
Blood cultures if suspected infection
HbA1c- Blood sugar over previous 3mths
TFTs and TPO (AI thyroid disease)
anti-TTG- Coeliac disease
Insulin antibodies, anti-GAD antibodies and islet cell antibodies
266
Outline basics of long term management of T1D
SC insulin regimes
Monitor dietary carbs
Monitor blood sugar levels waking/each meal/before bed
Monitor for and manage complications
267
Outline basal bolus regimes of insulin in T1D
Basal- Injection of long acting insulin (lantus) in evening- Constant background insulin throughout day
Bolus- Injection short acting insulin (actrapid)- 3x/d before meals
268
Outline tethered pumps
Replaceable infusion sets and insulin
Attached at belt with tube that connects from pump to insertion site
Controls for infusion on pump itself
269
Outline patch pumps
Sit directly on skin
When run out of insulin- Replace entire patch pump and new pump attached
Controlled by separate remote
270
Outline hypoglycaemia in T1D
Caused by too much insulin, not enough carbs or issues with absorption (diarrhoea, vomiting, sepsis)
271
What are the symptoms of hypoglycaemia?
Hunger
Tremor
Sweating
Irritability
Dizziness
Pallor
Reduced consciousness
Coma
Death
272
Outline management of hypoglycaemia
Rapid acting glucose (lucozade) and slower acting carbs (bread)
Severe:
IV glucose or IM glucose
273
What are the causes of hypoglycaemia?
T1D
Hypothyroidism
Glycogen storage disorders
GH deficiency
Liver cirrhosis
Alcohol and fatty acid oxidation defects (MCADD)
274
What is the management of hyperglycaemia in T1D?
If not in DKA:
Increase insulin dose
Can take several hrs
275
What are the long term macrovascular complications of T1D?
Coronary artery disease
Peripheral ischaemia- Poor wound healing, ulcers, diabetic foot
Stroke
HTN
276
What are the microvascular complications of T1D?
Peripheral neuropathy
Retinopathy
Glomerulosclerosis
277
What are the infection related complications of T1D?
UTIs
Pneumonia
Skin and soft tissue infections- Particularly feet
Oral and vaginal candidiasis
278
Outline monitoring of T1D
HbA1c every 3-6mths
Cap blood glucose
Yrly ophthalmology appointment
Flash glucose monitoring- 5min lag behind BM
279
Outline pathophysiology of DKA
Extreme hyperglycaemic ketosis
Not producing insulin/not injecting adequate replacement
Results in:
Ketoacidosis
Dehydration
Potassium imbalance
280
Outline ketoacidosis in DKA
Cells in body have no fuel- Initiate ketogenesis
Glucose and ketone levels increase
Initially kidneys produce bicarbonate to buffer ketone acids which use up bicarbonate- Blood becomes acidotic
281
Outline dehydration in DKA
Hyperglycaemia overwhelms kidneys and glucose filtered into urine drawing water out with it
Polyuria and polydipsia
282
Outline potassium imbalance in DKA
Insulin normally drives potassium into cells
No insulin- Serum potassium high/normal
Kidneys balance blood potassium- Excrete excess in urine
Total body potassium- Low
When treatment with insulin started- Severe hypokalaemia- Arrhythmias
283
What are patients with T1D in DKA at risk of developing?
Cerebral oedema
Dehydration and hyperglycaemia- Water moves out of brain cells
Rapid correction of this causes rapid shift of fluid into brain cells- Oedema
284
How is the risk of cerebral oedema in DKA management managed?
Monitor GCS during fluid resus
Slow IV fluids
IV mannitol and IV hypertonic saline
285
Outline presentation of DKA
Polyuria
Polydipsia
N+V
Weight loss
Acetone breath
Dehydration and subsequent hypotension
Altered consciousness
Symptoms of underlying trigger, eg: Sepsis
286
Outline diagnosis of DKA
Hyperglycaemia >11mmol/l
Ketosis >3mmol/l
Acidosis <7.3
287
Outline management of DKA
1. Correct dehydration over 48h
2. Give fixed rate insulin infusion
Prevent hypoglycaemia- IV glucose when blood glucose <14mmol/l
Add potassium to IV fluids- Monitor K+ closely
288
What is adrenal insufficiency?
Adrenal glands don't produce enough steroid hormones- Cortisol and aldosterone
289
Outline primary adrenal insufficiency
Addison's disease
Adrenal glands damaged
Reduced secretion cortisol and aldosterone
AI
290
Outline secondary adrenal insufficiency
Inadequate ACTH stimulating adrenal glands
Low levels of cortisol released
Result of loss/damage pituitary gland
Can be due to congenital hypoplasia of pituitary/surgery/infection/loss of blood flow/radiotherapy
291
Outline tertiary adrenal insufficiency
Inadequate CRH release by hypothalamus
Usually result of being on long term steroid causing suppression of hypothalamus
292
What are the features of adrenal insufficiency in babies?
Lethargy
Vomiting
Poor feeding
Hypoglycaemia
Jaundice
Failure to thrive
293
What are the features of adrenal insufficiency in older children?
Nausea and vomiting
Poor weight gain or weight loss
Anorexia
Abdo pain
Muscle weakness/cramps
Developmental delay or poor academic performance
Bronze hyperpigmentation- Addison's- High ACTH levels stimulating melanocytes
294
Outline investigations of adrenal insufficiency
U&Es- Hyponatremia and hyperkalaemia
Blood glucose- Hypoglycaemia
Test cortisol, ACTH, aldosterone and renin levels before giving steroids
295
Outline hormone results in Addisons disease
Low cortisol
High ACTH
Low aldosterone
High renin
296
Outline hormone results in secondary adrenal insufficiecy
Low cortisol
Low ACTH
Normal aldosterone
Normal renin
297
Outline the short synacthen test
ACTH stimulation test
Confirms adrenal insufficiency
Give synacthen (synthetic ACTH)- Blood cortisol measured at baseline, 30mins and 60mins
ACTH stimulates healthy adrenal glands to produce cortisol- Should double
298
Outline management of adrenal insufficiency
Hydrocortisone- Glucocorticoid- Replaces cortisol
Fludrocortisone- Mineralocorticoid- Replaces aldosterone
Steroid card and emergency ID tag
Increase dose during acute illness
299
Outline monitoring in adrenal insufficiency
Growth and development
BP
U&Es
Glucose
Bone profile
Vit D
300
Outline sick day rules
Dose of steroid needs increasing and given more regularly until illness resolved
Blood glucose monitored closely- Risk of hypoglycaemia
If D+V- IM injection steroid and likely admission for IV
301
What is an Addisonian Crisis?
Adrenal crisis
Acute presentation of severe Addisons
Reduced consciousness
Hypotension
Hypoglycaemia, hyponatremia, hyperkalaemia
302
Outline management of Addisonian crisis
Intensive monitoring
IV hydrocortisone
IV fluid resuscitation
Correct hypoglycaemia
303
What is congenital adrenal hyperplasia?
Caused by congenital deficiency of 21-hydroxylase enzyme
Underproduction of cortisol and aldosterone and overproduction of androgens
Autosomal recessive
304
What is the function of testosterone?
Androgen hormone
High levels in men, low levels in women
Promotes male sexual characteristics
305
Outline glucocorticoid hormones
Help body deal with stress
Raises blood glucose
Reduces inflammation
Suppresses immune system
CORTISOL
Released in response to ACTH from anterior pituitary
306
Outline mineralocorticoid hormones
Act on kidneys to balance salt and water in blood
ALDOSTERONE
Released by adrenal gland in response to renin
Acts on kidneys to increase sodium reabsorption into blood and increase potassium secretion into urine
Increases sodium and decreases potassium
307
Outline pathophysiology of congenital adrenal hyperplasia
Deficiency in 21-hydroxylase enzyme
Progesterone not converted to aldosterone or cortisol- Gets converted to testosterone instead
Low aldosterone and cortisol
High testosterone
308
Outline presentation of severe cases of CAH
Female- Virilised genitalia and enlarged clitoris
Shortly after birth- Hyponatremia, hyperkalaemia, hypoglycaemia
Poor feeding
Vomiting
Dehydration
Arrhythmias
309
Outline presentation of female patients with mild CAH
Tall for age
Facial hair
Absent periods
Deep voice
Early puberty
Skin hyperpigmentation- Increased ACTH due to low cortisol
310
Outline presentation of male patients with mild CAH
Tall for age
Deep voice
Large penis
Small testicles
Early puberty
Skin hyperpigmentation- Increased ACTH due to low cortisol
311
Outline management of CAH
Cortisol replacement- Hydrocortisone
Aldosterone replacement- Fludrocortisone
Corrective surgery offered if virilised genitalia
312
Outline the role of GH
Produced by anterior pituitary
Stimulates cell reproduction and growth of organs, muscles, bones, height
Stimulates release of IGF-1 by liver- Promotes growth in children
313
Outline pathophysiology of GH deficiency
Congenital:
Disruption to GH axis at hypothalamus or pituitary gland
Can be due to genetic mutation in GH1 or GHRHR genes or empty sella syndrome
Acquired:
Secondary to infection/trauma/surgery
314
Outline presentation of GH deficiency
Micropenis
Hypoglycaemia
Severe jaundice
Poor growth
Short stature
Slow development of movement and strength
Delayed puberty
315
Outline investigations of GH deficiency
GH stimulation test- Test response to meds that normally stimulate release of GH- Eg: Insulin, glucagon, arginine, clonidine- GH deficiency= Poor response
Test for thyroid and adrenal deficiency
MRI brain
Genetic testing- Turner syndrome and Prader-Willi syndrome
Xray or DEXA scan- Assess bone age
316
Outline treatment of GH deficiency
Daily SC injections somatropin (GH)
Close monitoring height and development
317
Outline congenital hypothyroidism
Child born with underactive thyroid
Screened for on newborn blood spot
318
How can congenital hypothyroidism present?
Prolonged neonatal jaundice
Poor feeding
Constipation
Increased sleeping
Reduced activity
Slow growth and development
319
What are the causes of acquired hypothyroidism?
AI thyroiditis (Hashimoto's)
320
Outline Hashimoto's thyroiditis
AI inflammation of thyroid gland and subsequent underactivity
Anti-TPO antibodies and antithyroglobulin antibodies
321
Which conditions are associated with Hashimoto's thyroiditis?
T1D
Coeliac disease
322
Which antibodies are associated with Hashimoto's thyroiditis?
Anti-TPO
Antithyroglobulin
323
What are the symptoms of Hashimoto's thyroiditis?
Fatigue and low energy
Poor growth
Weight gain
Poor school performance
Constipation
Dry skin and hair loss
324
Outline management of hypothyroidism
Investigate TFTs, thyroid US and thyroid antibodies
Levothyroxine OD
325
What is a hydrocele?
Collection of fluid within tunica vaginalis surrounding testes
326
What is a simple hydrocele?
Common
Fluid trapped in tunica vaginalis
Fluid reabsorbed over time, hydrocele disappears
327
What is a communicating hydrocele?
Tunica vaginalis connected with peritoneal cavity
Hydrocele fluctuates in size
328
Outline examination of hydrocele
Soft, non-tender swelling around one testes
Transilluminate with light
329
Outline management of hydrocele
US confirms diagnosis
Simple- Resolve in 2y
Communicating- Surgery to remove/ligate processus vaginalis
330
What is hypospadias?
Urethral meatus displace to underside of penis towards scrotum
Can be associated with chordee- Head of penis bends down
Congenital condition
331
Outline management of hypospadias
Do not circumcise until urologist confirms ok
Surgery after 3-4mths age
332
What are the complications of hypospadias?
Difficulty urinating
Cosmetic and psychological concerns
Sexual dysfunction
333
What is cryptorchidism?
Testes that have not descended out of abdomen
May be palpable in inguinal canal
334
What risks are associated with undescended testes?
Testicular torsion
Infertility
Testicular cancer
335
What are the risk factors for undescended testes?
FHx
Low birth weight
SGA
Prematurity
Maternal smoking during pregnancy
336
Outline management of undescended testes
Watch and wait- Normally descend in 1st 3-6mths
Orchidopexy between 6-12mths
337
What are retractile testicles?
Normal in boys not yet reached puberty for testes to move out of scrotum into inguinal canal when cold/cremasteric reflex activated
If fail to to descend following puberty- Orchidopexy
338
What is a posterior urethral valve?
Tissue at proximal end of urethra causes obstruction of urine output
Newborn boys
Causes back pressure in bladder, ureters and kidneys- Hydronephresis
Prevents full emptying
339
Outline presentation of posterior urethral valve
Difficulty urinating
Weak urine stream
Chronic urinary retention
Palpable bladder
Recurrent UTIs
Impaired kidney function
Severe- Bilateral hydronephresis and oligohydramnios
Oligohydramnios leads to underdeveloped fetal lungs and respiratory failure shortly after birth
340
Outline investigations of posterior urethral valve
341
Outline management of posterior urethral valve
Mild- Observe and monitor
Temporary urinary catheter to bypass valve
Definitive- Ablation/removal of extra urethral tissue (cystoscopy)
342
What is a Wilms’ tumour?
Specific tumour affecting kidney in children <5y
343
Outline presentation of Wilms’ tumour
<5y
Mass in abdomen
Abdo pain
Haematuria
Lethargy
Fever
HTN
Weight loss
344
Outline diagnosis of Wilms’ tumour
US of abdomen
CT or MRI- Staging
Biopsy- Definitive
345
Outline management of Wilms’ tumour
Surgical excision of tumour and affected kidney (nephrectomy)
Adjuvant chemo/radiotherapy
346
What is a multicystic dysplastic kidney?
One of baby's kidneys made up of multiple cysts, other normal- Good prognosis
Rarely bilateral- Causes death in infancy
Cystic kidney may atrophy and disappear <5y old
347
What is the risk of having a single kidney in later life?
UTIs
HTN
CKD
348
Outline diagnosis of multicystic kidney
Antenatal US scan
349
Outline management of multicystic dysplastic kidney
No treatment required
350
Outline autosomal recessive polycystic kidney disease (ARPKD)
Most common in children
Diagnosed on antenatal US
351
What are the causes of ARPKD?
Cystic enlargement of renal collecting ducts
Oligohydramnios, pulmonary hypoplasia, Potter syndrome
Congenital liver fibrosis
352
How does ARPKD present?
Antenatal- Oligohydramnios, polycystic kidneys
Lack of amniotic fluid leads to Potter syndrome and pulmonary hypoplasia
May require dialysis within 1st few days of life
Most develop end-stage renal failure before adulthood
353
List ongoing problems a patient with PKD may have throughout their life
Liver fibrosis and failure
Portal HTN- Oesophageal varices
Progressive renal failure
HTN due to renal failure
CLD
354
What is the prognosis of PKD?
Poor
Around 1/3rd survive to adulthood
355
What is enuresis?
Involuntary urination
Nocturnal- Bed wetting
Diurnal- Day wetting
Daytime control- 2y
Nighttime urination- 3-4y
356
Outline primary nocturnal enuresis
Never been consistently dry at night
357
What are the causes of primary nocturnal enuresis?
Overactive bladder- Frequent small volume urination prevents development of bladder capacity
Fluid intake prior to bedtime
Failure to wake during sleep
Psychological distress
Secondary causes- Chronic constipation, UTI, LD, cerebral palsy
358
Outline management of primary nocturnal enuresis
2wk diary
Reassure if <5y- Likely to resolve
Lifestyle changes
Encouragement and positive reinforcement
Treat underlying causes
Enuresis alarms
Pharmacological treatment
359
Outline secondary nocturnal enuresis
Previously been dry >6mths
360
List causes of secondary nocturnal enuresis
UTI
Constipation
T1D
New psychosocial problems
Maltreatment
361
Outline diurnal enuresis
Daytime incontinence
Dry at night but still episodes of urinary incontinence during day
362
List causes of diurnal enuresis
Urge- Overactive bladder
Stress- Leakage during stressors
Recurrent UTIs
Psychosocial problems
Constipation
363
Outline pharmacological management of enuresis
Desmopressin- Reduces volume urine produced by kidneys- Take at bedtime to reduce nocturnal enuresis
Oxybutynin- Reduces contractility of bladder in urge incontinence
Imipramine- TCA
364
Outline haemolytic uraemic syndrome (HUS)
Small thrombosis in small blood vessels throughout body
Triggered by Shiga toxins
Most commonly affects children following episode of gastroenteritis
ABs and anti-motility (eg: Loperamide) increase risk
365
Which bacteria can produce shiga toxin?
Shigella
E. coli
366
What is the classic triad of HUS?
Microangiopathic haemolytic anaemia
AKI
Thrombocytopenia (low platelets)
367
Outline presentation of HUS
1st symptoms- Diarrhoea- Bloody within 3d
1 wk later:
Fever
Abdo pain
Lethargy
Pallor
Reduced urine output (oliguria)
HAematuria
HTN
Bruising
Jaundice (due to haemolysis)
Confusion
368
Outline management of HUS
MEDICAL EMERGENCY
Stool culture
Supportive management:
Hypovolaemia- IV fluids
HTN
Severe anaemia- Blood transfusions
Severe renal failure- Haemodialysis
Self-limiting- Most patients fully recover with good care
369
Outline nephritis
Inflammation within nephrons of kidney
Reduction in kidney function
Haematuria
Proteinuria- Less than in nephrotic syndrome
370
What are the most common causes of nephritis?
Post-streptococcal glomerulonephritis
IgA nephropathy
371
Outline post-streptococcal glomerulonephritis
Occurs 1-3wks after beta-haemolytic strep infection (eg: Tonsillitis)
Causes AKI
Management- Supportive- Antihypertensives and diuretics
372
Outline IgA nephropathy
Related to HUS- IgA vasculitis
Teenagers or young adults
373
What is seen on a renal biopsy in IgA nephropathy?
IgA deposits
Glomerular mesangial proliferation
374
Outline management of IgA nephropathy
Supportive management of renal failure
Immunosuppressants- Steroids and cyclophosphamide- Slow progression of disease
375
Outline nephrotic syndrome
Basement membrane in glomerulus becomes highly permeable to protein- Leaks from blood into urine
Most common between 2-5y
376
Outline presentation of nephrotic syndrome
Frothy urine
Generalised oedema
Pallor
Deranged lipid profile- High cholesterol, triglycerides, low density lipoproteins
High BP
Hyper-coagulability- Increased tendency to form blood clots
377
Outline classic triad of nephrotic syndrome
Proteinuria (++)
Oedema
Low serum albumin
378
Outline causes of nephrotic syndrome
Most common- Minimal change disease
Secondary to intrinsic kidney disease:
Focal segmental glomerulosclerosis
Membranoproliferative glomerulonephritis
Secondary to underlying systemic illness:
HSP
Diabetes
Infection (HIV, hepatitis, malaria)
379
Outline minimal change disease
Most common cause of nephrotic syndrome in children
Can occur in healthy children w/o risk factors
Urinalysis- Small molecular weight proteins and hyaline casts
380
Which condition shows small molecular weight proteins and hyaline casts on urinalysis?
Minimal change disease- Nephrotic syndrome
381
Outline management of minimal change disease
Corticosteroids (prednisolone)
Prognosis good but may reoccur)
382
Outline management of nephrotic syndrome
High dose steroids (eg: Prednisolone)- 4wks then wean off over 8wks
Low salt diet
Diuretics- Treat oedema
Albumin infusions if severe hypoalbuminaemia
AB prophylaxis if severe
If steroid resistant- ACE-is and immunosuppressants
383
Outline complications of nephrotic syndrome
Hypovolaemia
Thrombosis- Clotting proteins lost in kidneys
Infection- Kidneys leak Igs
Acute/chronic renal failure
Relapse
384
What is vulvovaginitis?
Inflammation and irritation of vulva and vagina
Common in girls 3-10y
Less common after puberty due to oestrogen
385
Outline presentation of vulvovaginitis
Soreness
Itching
Erythema around labia
Vaginal discharge
Dysuria
Constipation
Urine dip- Leukocytes but no nitrites (often confused for UTI)
386
Outline management of vulvovaginitis
Avoid washing with soap and chemicals
Avoid perfumed or antiseptic products
Good toilet hygiene- Wipe front to back
Keep area dry
Emoolients- Sudacrem to soothe
Loose cotton clothing
Treat constipation and worms
387
What is acute pyelonephritis?
Infection UTI infection affects tissue of kidney
Can lead to scarring and reduced kidney function
388
What is cystitis?
Inflammation of bladder
Result of bladder infection
389
What are the symptoms of UTI?
Fever- May be only symptoms
Fever
Lethargy
Irritability
Vomiting
Poor feeding
Urinary frequency
Suprapubic pain
Dysuria
Incontinence
390
Outline diagnosis of acute pyelonephritis
>38 degrees
Loin pain or tenderness
391
Outline diagnosis of UTI
Urine dip
If nitrites and leukocytes- Send MSU to lab
392
Outline management of UTIs
All children <3mths with fever- Immediate IV ceftriaxone, full septic screen and LP considered
>3mths- Oral ABs if otherwise well
Features of sepsis or pyelonephritis- Inpatient IV ABs
Trimethoprim
Nitrofurantoin
Cefalexin
Amoxicillin
393
Outline investigations of recurrent UTIs
<6mths 1st UTI- Abdo US within 6wks or during if recurrent UTIs/atypical bacteria
Recurrent UTIs- Abdo US within 6wks
Atypical UTIs- Abdo US during illness
DMSA 4-6mths after illness- Assess for damage from recurrent/atypical UTIs
MCUG- Investigate atypical/recurrent UTIs <6mths old or VUR
394
What is vesico-ureteric reflux?
Urine has tendency to flow from bladder back to ureters
Predisposes patients to upper UTIs and subsequent renal scarring
Diagnosed by MCUG
395
Outline management of VUR
Avoid constipation
Avoid excessively full bladder
Prophylactic ABs
396
Outline surfactant
Surface tension- Attraction of molecules in liquid to each other, keeps alveoli closed
Surfactant- Produced by type II alveolar cells- Reduces surface tension- Increases lung compliance- Promotes equal expansion all alveoli during inspiration
Produced between 24-34wks gestation
397
Outline cardiorespiratory changes at birth
During birth- Thorax squeezed clearing fluid from lungs
Birth, temperature change, sound and physical touch stimulate baby's 1st breath
Adrenaline and cortisol released in response to stress of labour- Stimulate respiratory effort
1st breath- Expands alveoli, decreases PVR- Fall in pressure RA- LA greater pressure= Closure of foramen ovale (fossa ovalis)
Increased blood oxygenation- Drop in circulating prostaglandins= Closure of ductus arteriosus (ligamentum arteriosum)
Immediately after birth- Ductus venosus stops functioning as umbilical cord clamped- Structurally closes a few days later (ligamentum venosum)
398
What is hypoxic-ischaemic encephalopathy?
Extended hypoxia to brain in neonate
Potentially life-long consequences- Cerebral palsy
399
Outline principles of neonatal resuscitation
Warm baby
Calculate APGAR at 1, 5, 10mins
Stimulate breathing- Dry vigorously, neutral head position, check for obstruction
Inflation breaths:
2 cycles 5 inflation breaths
No response- 30 secs ventilation breaths
No response- Chest compressions
3:1
IV drugs and intubation considered
Possible HIE- Therapeutic hypothermia with active cooling
400
Outline APGAR
Appearance:
0- Blue/pale centrally
1- Blue extremities
2- Pink
Pulse:
0- Absent
1- <100
2- >100
Grimace:
0- No response
1- Little response
2- Good response
Activity:
0- Floppy
1- Flexed arms and legs
2- Active
Respiration:
0- Absent
1- Slow/irregular
2- Strong/crying
401
Outline delayed umbilical cord clamping
Placental transfusion
Improves Hb, iron stores and BP
Reduces risk of intraventricular haemorrhage and necrotising enterocolitis
Increases risk of neonatal jaundice
1min delayed cord clamping in uncompromised neonates
402
