Paeds emergencies, genetics and neonatology Flashcards
(289 cards)
1
Q
Recall 2 causes of neonatal collapse
A
Sepsis
CHD
2
Q
Recall 4 possible causes of jaundice in the neonate
A
Breast milk
Sepsis
Feeding difficulty
Physiological
3
Q
Recall 4 common causes of rash in the neonate
A
Nappy rash
Milia
Erythema toxicum
Mongolian blue spot
4
Q
Recall 2 causes of seizures in the neonate?
A
Hypoglycaemia
HIE
5
Q
Recall 4 milestones that should be reached by one year
A
Cruises
Pincer grip
“mama, dada, no”
Waves bye-bye
6
Q
Recall 5 milestones that should be reached by 18 months
A
Stack 2 blocks
walk unaided/ run
separation anxiety
know 6-12 words
scribbles
7
Q
Recall 3 milestones that should be reached by 2 years
A
Link 2 words in sentences
understand 2 step commands
Walks upstairs (2 feet/ step)
8
Q
Recall the milestones that should be reached by 3 years
A
GM: Hop on 1 foot, walk upstairs 1 foot per step, downstairs 2 feet per step
FM: Copies circle, bricks in a bridge, makes single cuts in paper with scissors, string of beads
S+L: understands negatives + adjectives
Social: begins to share toys, plays alone without parents, eats with fork + spoon, bowel control
9
Q
What are the 4 domains of development?
A
Gross motor skills (develop head to toe)
Fine motor skills
Language + speech
Social skills
10
Q
In what period of life should primitive reflexes be present?
A
From birth, to no later than 6 months
11
Q
Recall the 5 primitive reflexes
A
Moro (sudden head drop –> arms outstretched)
Stepping
Rooting
Palmar + plantar grasp
Atonic neck (fencing posture)
12
Q
Why do gross motor skills develop from head to toe?
A
That is how myelination develops
13
Q
At what age should a child run and jump?
A
2.5 years
14
Q
What is the limit age for walking independently?
A
18 months
15
Q
At what age should children babble polysyllabically?
A
6 months
16
Q
At what age should children say 6 words with meaning?
A
18 months
17
Q
At what age should children be able to smile?
A
6 weeks
18
Q
How can abnormal progression be classified?
A
Slow but steady
Plateau,
Regression
Acute insult
19
Q
What is tested in the full physical exam (first 72 hours)?
A
Heart
Hips
Eyes
Testes
20
Q
When is the new baby review done and what does it involve?
A
2 weeks: safe sleeping, vaccination, feeding, caring, development
21
Q
How is hearing tested in the neonate, and when is this done?
A
Automated Otoacoustic Emission/ AOAE
At 4 weeks
22
Q
When is the second full physical exam, and what does it involve?
A
At 6 weeks (done by GP) - DDH testing, testicles, heart, weight, length, vaccination discussion
23
Q
Recall the timings of the immunisations done within the 1st year of life and what is included in each one
A
1st: 2m: 6-in 1 DTaP/IPV/Hib/HepB, rotavirus, Men B
2nd: 3m: ‘6-in-1’, rotavirus + pneumococcal
3rd: 4m: 6-in-1 + MenB
4th: 1y: 1st MMR + boosters
24
Q
When is HBV given, and to which infants?
A
At birth, to those whose mother is infected
25
When is BCG given to eligible infants?
At birth
26
What is included in a health review?
Development
Behaviour
Healthy eating
27
When are the health reviews conducted?
1 + 2 years
28
When are the 5th immunisations and what do they include?
3 years 4 months
DTaP/IPV, 2nd MMR
29
What is checked at 4 years?
Vision
30
What is checked on school entry?
Height
Weight
Hearing
31
When are the 6th and 7th immunisations and what do they include?
6th: 12-13y: 2 x HPV (6,11,16,18)
7th: 14y, DTP, MenACWY
32
Which paediatric milestones are checked at the 1 year health review?
GM: should be walking unsteadily + standing independently
FM: pincer grip (check for no hand dominance)
Hearing/ S+L: 2/3 words other than dada/mama with intent
Social/emotional/ behavioural: drink from cup with 2 hands
33
What is the mnemonic for remembering the components of the 6-in-1 vaccine?
Parents Will Immunise Toddlers Because Death -
Polio
Whooping cough
Influenzae B
Tetanus
B (hepatitis)
Diptheria
34
What colours of skin would be a red flag in the traffic light system?
Pale/ mottled/ ashen/ blue
35
At what age is a child with fever always considered a red flag in the traffic light system?
<3 months
36
Recall how CPR differs in adults compared to children and neonates
Adults: 30:2
Children: 15:2
Neonates: 3:1
37
In the ABCDE formulation, what comes under 'disability'?
AVPUG - Alert, voice, pain, unresponsive, glucose
38
What is the most common surgical emergency in newborn babies?
Necrotising enterocolitis
39
Describe the decorticate and decerebrate positions
Decorticate = bending wrists up to neck
Decerebrate = wrists pointing out, arms straight down by sides
40
What is SIRS?
Generalised inflammatory response, defined by >/= 2 criteria:
Must inculde 1 of:
- Abnormal temp (<36, >38.5)
- Abnormal WCC
The other criteria are:
- Abnormal HR
- Raised RR
41
How is a high risk sepsis diagnosed?
CVS: hypotension, prolonged cap refil, O2 needed to maintain SpO2
Blood lactate >2
Pale, mottled or non-blanching purpuric rash
RR abnormal or grunting
42
What is the sepsis 6 pathway in adults?
Oxygen
Blood + blood cultures
IV Abx
IV fluids
Check serial lactates
Check urine output
43
What is the difference between Sepsis and SIRS?
Sepsis = SIRS with infection
44
How is severe sepsis defined?
Sepsis with CV dysfunction, ARDS or dysfunction 2 or more organs
45
How is septic shock defined?
Sepsis with CV dysfunction persisting after >, 40mL/kg of fluid resuscitation in 1 hour
46
What are the common organisms implicated in early onset neonatal sepsis?
GBS
E coli
L monocytogenes
47
Which organism is most likely to cause late onset neonatal sepsis?
Coagulase-negative staphylococcus (CoNS) eg. Staph. Epidermis
48
Which children with sepsis should have an LP?
<1 month old
1-3 months who appear unwell/ have WCC <5 or >15
49
What is the sepsis 6 pathway in children?
Give:
1. High-flow O2
2. Abx
- Early-onset neonatal = cefotaxime, amikacin + ampicillin
- Late-onset neonatal = meropenem + amikacin + ampicillin
- >3m old = ceftriaxone )
3. Early senior input
4. Early inotropic support
5. Fluid resus if indicated (20mls/kg 0.9% NaCl over 5-10 mins)
Take:
1. Bloods:
FBC (abnormal WCC?)
U&E + CRP (?urosepsis)
Glucose
Clotting (?DIC)
ABG + lactate
50
Which Abx are most useful in meningococcal sepsis?
IM benzylpenicillin (in community)
or
IV cefotaxime (in hospital)
51
Which Abx are most useful in early onset neonatal sepsis?
Most likely to be GBS, L. monocytogenes or E coli so:
IV cefotaxime + amikacin + ampicillin
52
Which Abx are most useful in late onset neonatal sepsis?
Most likely to be CoNS (s. epidermis) so:
IV meropenem + amikacin + ampicillin
53
What is opisthotonos?
Hyperextension of neck + back
54
What are the two 'signs' indicative of meningitis?
Kernig's sign: pain on leg straightening
Brudzinski's sign: supine neck flexion --> knee/ hip flexion
55
What type of rash is often present in meningitis and what type of meningitis is this most common in?
Non-blanching: meningococcal
56
How does the HR change throught the course of illness in meningitis?
Starts high to compensate for brain ischaemia, then drops to as baroreceptors sense high BP
57
What symptoms make up Cushing's triad of high ICP?
High BP
Low HR
Irregular RR
58
In what order should meningitis investigations be done?
LP if not CI to identify source of infection
1. VBG: inc. glucose + lactate
2. Blood cultures (BEFORE empirical abx started)
3. FBC, CRP, U+E and creatinine
(After this: give broad spec abx at highest possible dose without delay)
59
As well as sepsis 6 pathway + Abx, what should the management be in meningitis in children?
Steroids (dexamethosone) if CSF shows purulent CSF, WBC >10000, WCC + protein >1g/L, bacterial gram stain + ONLY if it's not meningococcal
Mannitol (to reduce ICP)
IV saline NaCl
60
What potential longterm complications of meningitis might need to be discussed with a child's family?
Hearing loss, renal failure, neurodevelopmental conditions
Purpura fulminans: haemorrhagic skin necrosis from DIC
61
What are the most common causes of viral meningitis?
Coxsackie Group B
Echovirus
62
What is encephalitis?
Inflammation of the brain parenchyma
63
What are the 3 possible aetiologies of encephalitis?
1. Direct invasion of cerebellum (eg HSV)
2. Post-infectious encephalopathy = delayed brain swelling following neuroimmunological response to antigen
3. Slow virus infection (eg HIV or SSPE following measles)
64
What are the signs and symptoms of encephalitis?
Same as meningitis: might not be able to tell the difference clinically! If behavioural change is more likely to be encephalitis
65
What are 6 contraindications for LP?
Cardiorespiratory instability
Signs of raised ICP
Thrombocytopaenia
Focal neurology
Coagulopathy
Meningococcal meningitis
66
How should encephalitis be managed?
IV acyclovir (high dose) for 3w
HSV is a rare cause but complications are major so treat empirically
67
What should be added to the treatment regime if it's a CMV encephalitis?
Ganciclovir + Foscarnet
68
What is anaphylaxis?
Type 1 hypersensitivity reaction
IgG cross-linking with IgE membrane-bound Ab of mast cell/ basophil
69
What is the most common cause of anaphylaxis in children?
Food allergy (85%)
70
What is the dose of IM adrenaline in paediatric anaphylaxis?
1:1,000
71
When can a repeat dose of IM adrenaline be given in paediatric anaphylaxis treatment?
If response after 5 mins is insufficient
72
After giving adrenaline, how should anaphylaxis be managed?
Establish airway + high flow O2
IV fluids (crystalloids)
IV chlorpheniramine
IV hydrocortisone
Salbutamol if wheeze
73
What is the first thing that must be done on observation of a dry baby at delivery?
Note time!
74
What must be done within the first 30 seconds of a neonatal resuscitation?
Assess tone, RR, HR (femoral + brachial) + colour
75
What must be done within the first 60 seconds of a neonatal resuscitation?
If not breathing, open airway, do 5 INFLATION BREATHS
Reassess + repeat until chest movement seen
76
Once chest movement is seen in a neonatal resuscitation, what should be done next?
Ventilate for 30s
Then chest compression + ventillation with a rate of 3:1
77
If HR remains undetectable/ slow in a neonatal resuscitation, what should be considered?
Consider venous access + drugs
78
When should the Apgar score be used?
At 1 + 5 mins after delivery, + every 5 mins after if condition remains poor
79
What apgar score is considered normal?
>7
80
What are the components of the apgar score?
Appearance (colour)
Pulse
Grimace
Activity (muscle tone)
Respiratory
81
What should be considered if, after tracheal intubation, HR does not increase and good chest movement is not achieved in a neonatal resuscitation?
DOPE:
Displaced tube
Obstructed tube
Patient (tracheal obstruction? Lung disorder? Shock? Choanal atresia?)
Equipment failure
82
When should 5 rescue breaths be given in paediatric BLS?
DR AB RESCUE BREATHS CDE
83
At what BPM should chest compressions be done in paediatric BLS?
100-120
84
Recall the signs and symptoms of Patau's
Use mnemonic 'microcephaly':
M: mental retardation
13 (trisomy)
C: cleft lip + palate
R: renal
O: omphalocele
C: cardiac
E: eyes small
P: polydactyly
H: holoprosencephaly
L: lbw
85
Recall some key features of Edward's syndrome
Use mnemonic CrIEs of SORROW
C: cardiac
I: intellectual disability
E: ears low-set
S: small mouth + chin
O: overlapping fingers
R: renal
R: Rocker-bottom' feet
O: omphalocele
W: lbw
86
Recall 3 key features of Down's
Single palmar crease
'sandal gap' abnormality
upslanting palpabral fissures
87
What is mutated in Noonan's syndrome?
RAS/ MAPK
88
Recall 5 features of Noonan's
Webbed neck
Trident hairline
Pectus excavatum
Short stature
Pulmonary stenosis
89
What is the genetic difference between PWS and Angelman's?
PWS = lack paternal 15q
Angelman = lack maternal 15q
90
What is the genetic mutation in Turner's?
Female missing/ partly missing an X chromosome
91
Recall 6 features of Turners
Neonatally: pyloric stenosis + cardiac problems
Infertility due to ovarian dysgenesis
Koilonychia
Wide carrying angle
Webbed neck
Bicuspid aortic valve --> aortic coarctation --> ESM over aortic valve
92
Recall 4 features of Kleinfelter's
Infertility
Hypogonadism
Gynaecomastia
Tall stature
93
What mutation causes fragile X?
CGG trinucleotide expansion - FMR1 gene
94
Recall the symptoms of Fragile X
MALE MOPS
M: macrocephaly
A: autism
L: laxity of joints
E: ears are large + low-set
M: macro-
O: orchidism
P: prolapsed MV complication
S: scoliosis
95
What is the triad of abnormalities seen when there is maternal rubella during pregnancy
Cataracts
Deafness
Cardiac abnormalities
96
What is the prognosis for Patau's syndrome?
80% die in first month
97
What is the prognosis for Noonan's?
Varies massively as penetrance varies massively too
98
What is the phenotype of Angelman's?
Cognitive impairment
Ataxia
Epilepsy
Abnormal facial appearance
99
What is the most common heart defect in Down's syndrome?
AVSD
100
Recall some of the possible later complications of Down's syndrome
Secretory otitis media (75%)
OSA (50-75%)
Learning difficulty
VI Joint laxity
101
Recall 5 conditions that those with Down's are at an increased risk of developing
AML
Hypothhyroidism
Coeliac
Epilepsy
Early-onset alzheimer's
102
What should be checked for annually in those with Down's syndrome
Hearing test
Thyroid
Eye test
Hb (for IDA),
103
What classifies as a stillbirth (rather than a miscarriage)?
Foetus born with no signs of life >24 weeks of pregnancy
104
What is the difference between the perinatal and neonatal mortality rate?
Perinatal = stillbirths + deaths within 1st week
neonatal = deaths of live-born infants in first 4 weeks after birth
105
For how long is a baby considered a neonate?
Up to 28 days old
106
What is considered 'term'?
37-41 weeks old
107
Recall the cutoff rates for low, very low and extremely low birthweight
Low = <2500g
Very low = <1500
Extremely low = <1000
108
How is small/large for gestational age calculated?
Small = in <10th centile
Large = >90th centile
109
What does a routine neonate inspection exam include?
Head to toe systematic:
1. Birthweight + gestational age
2. General observation - posture? Pallor? Rash?
3. Head (many things to look for)
4. Breathing, HR + peripheral saturations
5. Femoral pulses + genitalia
6. Musle tone, DDH, whole of back + spine, DDH/ Club feet?
7. Reflexes
110
What is being looked for upon examination of the neonatal head ?
Circumference (macrocephaly?)
Eyes (red reflex?)
Cephalohematoma (benign, self-resolving)
Caput Succedaneum = Cross Suture lines (self-resolving)
Tense fontanelle (raised ICP?)
Depressed fontanelle (dehydrated?)
111
What are the components of the test for DDH?
Barlow's (abduct + push joint posteriorly)
Ortolani's (lift + externally rotate)
112
What is the Guthrie test?
Biochemical screening
113
When should the Guthrie test be done?
At 7 days old
114
What is included in the Guthrie test?
Congenital hypothyroidism
SCD
CF
6 metabolic diseases:
- PKU
- MCADD
- Maple Syrup Urine disease
- IVA (isovaleric acidaemia)
- Glutaric aciduria type 1
- HCU (homocysteinuria )
115
Which type of infection can cause sensorineural hearing loss in neonates?
CMV
116
What is the 1st and 2nd line options for testing hearing at birth?
1st line: Evoked otoacoustic emission- If earphone doesn't produce an echo...
2nd line: Automated auditory brainstem response- Computer analyses the EEG wave forms evoked in response to a series of clicks
117
What features of FAS would be seen in the neonatal examination?
Microcephaly
Absent philtrum
Cardiac abnormalities
Reduced IQ
IUGR
Small upper lip
118
What is the triad of features seen in Rubella syndrome?
Cataracts
Deafness
Cardiac abnormalities
119
What abnormalities would be seen in a baby who is born to a mother with syphillis?
Saddle nose
Rhinitis
Deafness
Hepatosplenomegaly
Jaundice
120
What is the NIPE and when should it be done?
Neonatal + infant Physical Examination
<72 hours AND 6-8 weeks
121
What are the 4 major areas of the NIPE?
Heart, Eyes, Testes, Hips
122
What is positional talipes, and what causes it?
Feet remaining in in-utero position, due to intrauterine compression
123
How should positional talipes be managed?
Physiotherapy
124
What is club foot known as medically? How should club feet be managed?
Talipes equinovarus
Ponsetti method (plaster casting + bracing)
Surgery if severe
125
What is the cause of hypoxic ischaemic encephalopathy ?
Perinatal asphyxia - may be due to placental abruption, interruption of umbilical blood flow (eg shoulder dystocia --> cord compression), maternal hypotension, IUGR or failure to breathe at birth
126
If hypoxic ischaemic encephalopathy continues post-natally, what will it cause?
Cerebral palsy
127
What are 3 key features of mild hypoxic ischaemic encephalopathy ?
Staring eyes
Hyperventilation
Hypertonia
128
What are 3 key feature of moderate hypoxic ischaemic encephalopathy ?
Cannot feed
Possible seizures
Hypotonic
129
What are the key features of severe hypoxic ischaemic encephalopathy ?
No normal movement in response to pain
Tone fluctuates between hypertonic + hypotonic
Seizures refractory to tx
MODS
130
How should hypoxic ischaemic encephalopathy be managed?
Supportively
Resp support, anticonvulsants, fluid restriction (transient renal impairment), inotropes (to treat hypotension), therapeutic hypothermia (requires NICU)
131
At what age does brain injury classify as 'acquired brain injury' and not cerebral palsy?
2 years old
132
How can cerebral palsy be aquired post-natally?
Perventricular leukomalacia secondary to ischaemia (eg meningitis, very prem birth)
133
Recall some features of abnormal posture/tone due to cerebral palsy
Stiff legs which scissor
Hypotonia or spasticity
Inability to lift head
Rounded back when sitting
Fisted hands
134
Other than postural/ limb tone abnormalities, what 3 abnormalities might be seen in cerebral palsy?
Feeding difficulties
Abnormal gait
Hand preference before 1y
135
How is gross motor function classified?
GMFCS (gross motor funtion classification system!)
136
What are the subtypes of cerebral palsywith their relative prevalences?
Spastic CP (90%)
Dyskinetic CP (6%)
Ataxic CP (4%)
137
What is the cause of spastic cerebral palsy?
Damage to UMN pathway (pyramidal tracts)
138
What is one of the pathognomonic features of spastic cerebral palsys opposed to other types of cerebral palsy?
Clasp knife rigidity - increased tone suddenly gives way under pressure
139
What are the 3 main types of spastic cerebral palsy?
1) unilateral/ hemiplegia:
- unilateral arm + leg, face spared
- tiptoe walk on affected side
- likely to have an unremarkable Mx + birth
2) BL/ quadriplegia: all 4 limbs, often severe
- poor head control --> low central tone + seizures - often have LD
3) Diplegia (legs > arms affected)
- Abnormal walk
- A/w pre-term birth damage
140
What are the possible causes of dyskinetic cerebral palsy?
HIE or kernicterus
141
What is kernicterus?
Brain damage due to hyperbilirubinaemia
142
Describe the phenotype of dyskinetic cerebral palsy
Variable muscle tone predominated by primitive motor reflexes:
- Chorea
- Athetosis (fanning fingers)
- Dystonia (twisted appearance)
143
What is the cause of ataxic cerebral palsy?
Damage to cerebellum - most geneticly determined
144
How should cerebral palsy be investigated?
History, clinical examination and developmental progress - if cause is not clear, MRI
145
What one clinical sign is always an indication for referral for cerebral palsy investigation?
Persistent toe walking
146
What are some red flags for other neurological conditions when considering a diagnosis of cerebral palsy?
Absence of other RFs
FH of progressive neurological disorder
Loss of already attained cognitive/ developmental abilities
MRI suggestive of progressive neurological disorder
147
How should cerebral palsy be managed?
Very MDT orientated
- Info about prognosis: eg children who can sit by age 2 are likely to be able to walk unaided by 6
- SCOPE disability charity
- Medication: stiffness = diazepam, sleeping = melatonin, constipation = movicol, drooling = anticholinergic
148
Recall some important things to say in PACES counselling of a cerebral palsy child's parents
Explain brain damage has occurred in early development
Explain damage doesn't get worse, but will change its manifestation as the child gets older
Refer to MDT - esp. paediatrician
Long term Mx = physiotherapy, SALT + SEN
Medication options
149
Which babies are at highest risk of developing NEC?
Premature/ LBW
150
What is the aetiology of NEC?
Mostly unknown but thought to be a combination of poor blood flow + infection
151
What are the early signs of NEC?
Biliary vomiting (green)
Feeding intolerance
152
What will be seen on AXR in NEC?
Gas cysts
153
What are the appropriate investigations to do in NEC?
AXR
Blood cultures
154
What are the criteria used to decide management of NEC?
Bell's staging
155
Recall the elements of NEC management
1. Bowel rest: stop oral feed + switch to parenteral nutrition
2. Broad-spectrum Abx (eg Tazocin): duration depends on stage
3. Laparotomy (if perforation is seen on AXR)
156
Give some reasons for physiological jaundice at birth
1. Hb release from RBCs as there is high [Hb] at birth
2. RBC lifespan being 70d rather than 120d
3. Breast milk jaundice (but not until after >24h)
4. BR metabolism being less efficient in first few days of life
157
What is the main danger of uBR buildup in neonates?
Kernicterus (a form of encephalopathy): it's caused by a deposit of uBR in the basal ganglia
May develop into dyskinetic CP, LD + sensorineural deafness
158
How can uBR buildup in neonates be treated before any damage is done?
Phototherapy +/- IvIG and exchange transfusion
159
What would be the difference in clinical presentation between uBR buildup and cBR buildup?
uBR buildup --> kernicterus
cBR buildup --> dark urine + pale stools
160
In what situations should phototherapy be stopped?
If bronzing occurs: it means the child has a buildup of cBR, rather than uBR, which cannot be treated using phototherapy
161
How should investigations begin in neonatal jaundice?
1. Check transcutaneous or serum BR levels
2. Do a split BR to check uBR/cBR
3. May want to do a blood film analysis
162
Recall some pathological causes of neonatal jaundice in babies <24 hours old
GRAPHIC DOG
- Gilbert's
- Rhesus disease
- ABO incompatability (--> haemolysis)
- PK deficiency
- Hereditary spherocytosis
- Infection
- Crigler-Najjar
- Dubin-Johnson
- Other...
- G6PD deficiency
163
Recall 3 physiological causes of jaundice in a 2 day to 2 week old baby
Physiological jaundice
- due to immature liver, peaks at 3-5 days
Breastfeeding jaundice
- less milk intake --> more enterohepatic recycling
Breastmilk jaundice
- Decreased UGT1A1 activity (occurs following physiological jaundice)
164
What haemolytic and metabolic causes for jaundice might present in a 2 day to 2 week old baby?
Metabolic = Gilbert's, Crigler-Najjar, Dubin-Johnson
Haemolytic: G6PDD, PK deficiency, hereditary spherocytosis (less likely to be ABO at this point)
165
What may be the cause of jaundice in a 2 day to 2 week old baby that didn't present in first 24 hours?
Congenital hypothyroidism
Dehydration
Bruising (cephalohematoma)
Polycythaemia
166
How should jaundice be investigated in a baby over 2 weeks old?
Direct and indirect serum BR
167
Which of the conditions that cause jaundice in a 2 day to 2 week old baby might continue to the >2 weeks stage?
All - but physiological + breastmilk is most common
168
Recall 2 further causes of a buildup of uBR in a baby over 2 weeks old
Pyloric stenosis (presents at 2-4 weeks)
Congenital hypothyroidism*
169
Systematically recall the causes of a raised cBR in the neonate (>2w old)
Endocrine: Congenital hypothyroidism
GI: Billiary atresia, ascending cholangitis (can be caused by lipids on TPN)
Metabolic: Gal-1-PUT, A1AT deficiency, Tyrosinaemia type 1, peroxisomal disease
Other: CF, idiopathic neonatal hepatitis
170
For how long does jaundice have to persist in order to be defined as 'prolonged'?
>14 days if term
>21 days if pre-term
171
At what age should investigations include a split BR rather than just a total BR?
2 weeks
172
What is a worrying BR in a baby >37w gestation (red flag for kernicterus)?
>340
173
Recall the clinical features of kernicterus
Poor feeding, extreme letahrgy, hypotonia, high-pitched cry
174
Recall some investigations you might do to find the underlying cause of jaundice in a baby
TC/ serum BR within 6 hours of presentation
Haematocrit
DAT/ Coombs (haemolysis?)
G6PD levels (depending on ethnic origin)
TSH (hypothyroid?)
LFTs (ascending cholangitis? Biliary atresia?)
Blood group of M and B (ABO incompatible? Rhesus?)
Blood film + osmotic fragility (hereditary spherocytosis?)
MC&S of urine/ CSF (if ? infection cause)
175
How is treatment of neonatal jaundice guided?
There are thresholds at which phototherapy/ exchange transfusion are indicated
176
What are the options for treatment of neonatal jaundice?
Phototherapy +/- IV Ig
Exchange transfusion + phototherapy +/- IV Ig
177
How does phototherapy work?
Converts uBR to a water-soluble pigment that is excreted in urine
178
What important checks should be done during/ after phototherapy?
During: temperature, BR levels every 4-6 hours (with regular feed breaks)
After (12-18 hours post): check for a rebound hyperbilirubinaemia
179
When should intensive phototherapy be given?
1. Rapidly rising BR
2. Serum BR within 50mmol of exchange tranfusion threshold (after 72 hours life)
3. BR level doesn't respond after 6 hours of therapy
180
What are the 2 indications for exchange transfusion to treat neonatal jaundice?
1. BR threshold reached
2. Signs of kernicterus
181
What is an important thing to remember when giving an exchange transfusion?
Give folic acid afterwards to prevent anaemia
182
What is one thing to be cautious of if delivering high oxygen levels to a neonate?
Retinopathy of prematurity
183
Recall 6 signs of respiratory distress in babies
High RR (>60)
Laboured breathing
Chest wall recessions
Nasal flaring
Expiratory grunting
Cyanosis (if severe)
184
What is the most common cause of respiratory distress in term infants?
Transient tachypnoea of the neonate
185
Recall 4 conditions that PPHTN can be secondary to
Birth Asphyxia
Meconium aspiration
Septicaemia
RDS
186
What is the pathophysiology of PPHTN?
High pulmonary vascular resistance --> right to left shunting within lungs at atrial and ductal levels
187
Recall some signs and symptoms of PPHTN
Cyanosis after birth
Absent heart murmurs + signs of HF
188
What investigations are appropriate in PPHTN and what would they show if pos?
CXR: normal heart size but some pulmonary oligaemia
Echo: to ensure no cardiac defect
189
How should PPHTN be treated?
Oxygen, NO (inhaled), sildenafil (!!)
Ventilation: mechanical, high frequenct (oscillatory)
OR, if severe, extracorporeal membrane oxygenation (ECMO) +/- heart/lung bypass
190
What is the cause of TTotN?
Delay in resorption of lung fluid
191
How is a diagnosis of TTofN made?
1. CXR - shows fluid in horizontal fissure
2. Other causes excluded
192
What are the possible causes of paediatric chronic lung disease?
Infection, barotrauma, iatrogenic injury
193
What is the pathophysiology of paediatric chronic lung disease?
Lung damage due to pressure and volume trauma from artificial ventilation, O2 toxicity and infection
194
What are appropriate investigations in paediatric chronic lung disease and what would pos results show?
CXR: widespread opacification
CBG/VBG: acidosis, hypercapnia, hypoxia
195
How can paediatric chronic lung disease be managed?
Respiratory support (!): prolonged artificial ventilation --> wean to CPAP --> wean to additional O2
Corticosteroid therapy - dexamethosone is useful for short-term clinical improvement (but concerns limit use)
196
What is the cause of RDS in neonates?
Deficiency of surfactant (common if born <28w gestation)
197
Recall 2 risk factors for RDS
DM mother
2nd born of premature twin
198
How is RDS diagnosed?
Clinical dx
Can be supported by:
- Pulse oximetry
- CXR showing pneumothorax (from ventilation), ground-glass appearance
199
How can RDS be managed antenatally and postnatally?
Antenatal: steroid therapy + tocolytic therapy so steroids have at least 24h to work
Postnatal: oxygen + ventilation (caution: CLD), CPAP
200
What is the cause of pneumothorax in children, and what is the best way to prevent them?
Ventilation which causes pulmonary interstitial emphysema
Can be prevented that infants are ventilated on the lowest possible pressures with adequate chest movement and blood gaes
201
How should infant pneumothorax be treated?
Immediate decompression
Oxygen therapy
Chest drain if tenion pneumothorax
202
When does meconium aspiration occur?
Exclusively in immediate neonatal period
203
What is the biggest RF for meconium aspiration?
Increased GA
204
What are 3 signs and symptoms of meconium aspiration?
Respiratory distress
Chest retraction
Hypoxia
205
How is meconium aspiration diagnosed?
CXR: overinflated lungs, patches of collapse + consolidation
May show pneumothorax or pneumomediastinum (from air leak)
206
How is meconium aspiration managed?
Observation: meconium-stained amniotic fluid + no hx of GBS
IV ampicillin + IV gentamicin (to treat features of infection)
CPAP (for severe cases)
207
What is meconium ileus?
Thick, sticky meconium that has a prolonged passing time
208
What is the normal period of delivery of meconium?
Within 24h
209
Recall 2 associations of meconium ileus
CF
Biliary atresia
210
How should meconium ileus be managed?
1st line: Gastrograffin enema
2nd line: surgery
211
Recall 4 differentials for billious vomiting in the neonate
NEC
Duodenal/ jejunal/ ileal atresia
Meconium ileus
Malrotation volvulus
212
What is the most important investigation to do in investigating billious vomiting, and what results might it show?
AXR: "Double bubble" sign = duodenal atresia
Air-fluid levels: jejunal/ ileal atresia
Dilated bowel loops --> NEC
213
How are duodenal/ jejunal/ ileal atresia managed?
Duodenal atresia --> duodenoduodenostomy
Jejunal/ ileal atresia --> laparotomy
214
How is malrotation volvulus managed?
Ladd's procedure
215
When does malrotation volvulus present, and how is it diagnosed?
3-7 days
Upper GI contrast or USS
216
What is the pathophysiology of cleft lip/ palate?
Failure of fusion of the frontonasal and maxiliary processes
217
Recall one condition that babies with cleft lip/ palate are at an increased risk of
Secretory otitis media
218
Recall 2 maternal risk factors for cleft lip/ palate
Antiepileptic/ BDZ use
219
What are some pre-surgical concerns to bear in mind in cleft lip/ palate?
Specialised feeding
Watch out for aiway problems
Pre-surgical lip-tapping/ nasal alveolar modelling to narrow cleft
220
What is the name for a left-sided diaphragmatic hernia?
Bochdalek hernia
221
When does diaphragmatic hernia form in utero?
At around 6-8w gestation
222
Recall 2 factors affecting prognosis in diaphragmatic hernia
1. Liver position
2. Lung-to-head ratio
223
What are the signs and symptoms of diaphragmatic hernia?
Respiratory distress at delivery
Concave chest at birth
224
How is diaphragmatic hernia diagnosed?
Routine USS following resp distress at birth
225
What 3 features does CXR show in diaphragmatic hernia?
mediastinum displaced to left
collapsed left lung
bowel loops in thorax
226
Recall the 1st and 2nd line management options for diagphragmatic hernia management
1st: NG tube + suction - prevents distention of intrathoracic bowel + allows breathing
2nd: Surgical reduction + repair, allowing re-expansion of the lung
- TPN/ ventilation needed for recovery
227
What is oesophageal atresia?
Malformation of the oesophagus so that it does not attach to the stomach
228
What is tracheo-oesophageal fistula?
Part of the oesophagus is joined to the trachea - often occurs alonsgide OA
229
What is the main risk of oesophageal atresia/ tracheo-oesophageal fistula?
Stomach acid can regurgitate into the lungs causing CLD/ BPD (bronchopulmonary dysplasia)
230
How will amniotic fluid be affected by a TOF that is preventing swallow?
Polyhydramnios
231
How should tracheo-oesophageal fistula/ oesophageal atresia be investigated?
NG tube to aspirate the stomach contents can quickly confirm or exclude
Gold standard = gastragaffin swallow
232
Recall the management of oesophageal atresia/ tracheo-oesophageal fistula
1. Replogle tube to drain saliva from oesophagus
2. Surgical repair (within a few days of birth) followed by NICU and ventilator support
233
What is biliary atresia?
Progressive fibrosis + obliteration of extra- + intra-hepatic trees, leading to chronic liver failure in 2 years
234
What are the subtypes of biliary atresia?
T1 - common bile duct atresia
T2 - cystic duct atresia
T3 - full atresia (>90%)
235
What are the signs and symptoms of bililary atresia?
Obstructive jaundic picture (pale stools, dark urine) with no vomiting
Hepatosplenomegaly
Normal BW --> faltering growth
236
Once a raised cBR has been identified, how can biliary atresia be diagnosed? What, then, is the gold standard?
USS: triangular cord sign
LFT: raised GGT
Gold standard: TIBIDA isotope scan (radioisotope scan of bile secretion) confirmed by ERCP + biopsy
237
What is the 1st-line management of biliary atresia?
Kasai hepatoportoenterostomy
Ligate fibrous ducts abive the join with the duodenum
Join an end of the duodenum directly to the porta hepatis of the liver
If unsuccessful --> transplant
238
How are the complications of biliary atresia managed?
Fat-soluble vitamins (as bile ducts affected)
Usodeoxycholic acid (this promotes bile flow)
Prophylatic Abx (to prevent cholangitis - cotrimoxazole)
239
What is small bowel atresia?
Congenital absence or complete closure of part of the lumen of the small bowel
240
Recall 3 associations of duodenal atresia
Congenital cardiac abnormalities
Down's
Polyhydramnios
241
What are the signs and symptoms of small bowel atresia?
Bile-stained vomiting is the main one
If non-bilious may be duodenal rather than jejunal/ ileal
Abdominal distention
242
How should small bowel atresia be managed?
ABCDE to stabilise neonate+/- NG tube decompression
Surgical:
- primary anastomosis or LADD procedure if malrotation is present
- Need to examine the whole bowel while you're at it to exclude other multiple atretic segments
243
What is the main gene implicated in CAKUT?
PAX 2
244
Recall 4 renal conditions of CAKUT
Multicystic kidneys
Renal agenesis
Medullary sponge kidney
Horseshoe kidney
245
Recall 3 non-renal conditions of CAKUT
Pelvouteric junction obstruction
Vesicoureteral reflux (in 30% of children presenting with UTIs)
Bladder outlet obstruction
246
Recall some antenatal signs of CAKUT
Oligohydramnios
Decreaed foetal UO
247
Recall some postnatal signs of CAKUT
Often present with UTI
Intra-abdominal mass
Haematuria
Renal calculi/ renal failure
Hepatosplenomegaly
248
What is Potter's sequence?
BL renal agenesis
Abnormal facies (widely separated eyes, low set ears, receding jaw)
Caused by OLIGOHYDRAMNIOS
249
Recall five important investigations you could do when looking for a cause of CAKUT
Renal USS
DMSA scan (Tc-99) - detects scarring + functional defects
MCUG (micturating cystourethrogram) - visualises anatomy (would see VUR)
MAG3 renogram (Tc-99) - dynamic screen shows MAG3 being excreted into urine using furosemoide
Genetic karyotyping
250
What are 6 signs of an atypical UTI?
Poor UO
Sepsis
Abdo/bladder mass
Failure to respond in <48 hours
Raised creatinine
Infection with non-E coli organisms
251
How do you choose which investigation to use in UTI?
If atypical, recurrent, or first UTI
<6m: Renal USS
If recurrent or atypical UTI <3 years old: DMSA scan
If VUR is suspected on USS, obstruction or trauma: MCUG/ VCUG
252
What is the pathophysiology of VUR?
The ureters enter the bladder perpendicularly --> shorter intramural course --> VUR
253
What is the main factor affecting prognosis of VUR?
Whether renal cause or not:
renal causes= bad prognosis
non-renal causes= good prognosis if treated
254
What are the types of anorectal malformation, and how can you tell the difference between them?
Low anorectal anomaly: anus closed over, in a different position or narrower than usual + fistula to skin
High anorectal anomaly: bowel has closed end at high level, not connecting with anus, fistula is usually to bladder/ urethra/ vagina
255
What are 4 symptoms of anorectal malformation?
Absent/ delayed meconium
Swollen abdomen
Vomiting
If there's a fistula they may pass stool from an abnormal area
256
How should anorectal malformation be investigated?
Checked on neonatal check - it's a clinical diagnosis
257
How is anorectal malformation managed in babies?
Surgical correction by 9 months
258
What is the normal period of time in which the testes descend?
Usually by 3m but can be up to 6m
259
At what point should a referral to a paediatric surgeon be made, if there is UL testicular undescent?
3 months
260
At what point should a referral to a paediatric surgeon be made, if there is BL undescent of testes?
May have a pituitary cause so immediately refer to paeds/ endo
261
What are the medical and surgical management options for cryptorchidism?
Medical: beta-hCG (may or may not be given)
Surgical: orchidopexy
262
What is billious vomit a red flag for?
Intestinal obstruction (intussusception, malrotation, strangulated inguinal hernia)
263
What is haematemesis a red flag for?
Oesophagitis/ PUD
264
What is projectile vomit at 2-7 weeks a red flag for?
Pyloric stenosis
265
What is vomiting at end of paroxysmal coughing a red flag for?
Whooping cough
266
What is abdo distention a red flag for in a baby?
Intestinal obstruction/ strangulated inguinal hernia
267
What is hepatosplenomegaly a red flag for?
Chronic liver disease, inborn error of metabolism
268
What is blood in stool a red flag for?
Intussusception, gastroenteritis (salmonella, campylobacter)
269
What are bulging fontanelles/ seizures a red flag for?
Raised ICP
270
What is a failure to thrive a red flag for?
GORD, coeliac, chronic GI
271
What is chronic vomiting in an infant most likely to be due to?
GORD or a feeding problem
272
What is transient vomiting in a child most likely to be due to?
Gastroenteritis, URTI
273
What needs to be excluded urgently when an infant is vomiting?
Meningitis and UTI
274
What is a failure to pass meconium in first 24 hours a red flag for?
Hirschprung's
275
What is FTT/ growth failure a red flag for in a constipated child?
Hypothyroid/ coeliac
276
What is gross abdo distention with constipation a red flag for?
Hirschprung's/ other GI dysmotility
277
What is abnormal LL neurology/ deformation a red flag for?
Lumbosacral pathology
278
What is a red flag for spina bifida?
Sacral dimple above nasal cleft
279
What is perianal fistula/ abscess/ fissure a red flag for in infants?
Perianal Crohn's
280
What is the normal feed pattern in newborns?
45-90ml per 2-3 hours
281
What is the normal feed pattern in a 2 month old?
120-150mls per 3/4 hours
282
What is the normal feed pattern in a 6 month old?
Solids should be being introduced
283
By what age should bowel habit be similar for a child as an adult?
4 years
284
Recall the most common complication of long-standing constipation in children
Overdistention --> lose feeling needed to defaecate --> involuntary soiling with overflow
285
How can constipation be treated in children?
Stool softeners (eg movicol)
If unsuccessful consider stimulant laxatives (eg senna)
Movicol can be used as a maintenance therapy
286
How is constipation diagnosed in a child?
2+ of the following:
- <3 complete stools per week
- Hard, large stool or 'rabbit dropping'
- Overflow soiling 1+ year
- Distress, pain, bleeding associated with stool
287
Recall the management protocol for paediatric status epilepticus
1. ABC
2. Check blood glucose - if <3mmol/L --> IV glucose and then re-check
3.
If vascular access --> IV lorazepam
If no vascular access --> PR diazepam/ buccal midazolam
4.
If IV access: More lorazepam
If still no IV access - PR paraldehyde
5.
If previously had IV access to give IV lorazepam but it hasn't worked, try PR paraldehyde
6.
If no response in *10 mins* call for senior help
If not on oral phenytoin: phenytoin
If already on oral phenytoin: phenobarbital
7.
If no response within 20 mins, call anaesthetist/ intensivist -->
Induction with thiopental
Mechanical ventilation
--> PICU
288
What is the dose per kg of lorazepam to give to children in status epilepticus?
0.1mg/kg
289
What is the dose per kg of diazepam to give to children in status epilepticus?
0.5mg/kg
