Neonates, Infection, Haematology, Oncology, Cardiology Flashcards
1
Q
Neonate/Newborn age
A
Neonate up to first 28 days
Newborn 0-2 months
2
Q
Infant age
A
2 months- 1 year
3
Q
Toddler age
A
1-2 years
4
Q
Premature gestation
A
<38 weeks
5
Q
Average weight of a newborn
A
- 5kg
7. 5 pounds
6
Q
Meconium passage
A
24-48 hours post-delivery
Very dark green colour
7
Q
APGAR score
A
Appearance Pulse Grimace Activity (Tone) Respiratory effort <3= Very low 7+= Normal
8
Q
Guthrie Heel Prick
A
1 week
PKU, Hypothyroidism, CF, Haemaglobinopathies
9
Q
When is Resus generally given to neonates?
A
> 23 weeks gestation
albeit it is remarkable if a <28 week survives
10
Q
Mechanisms to support a premature neonate
A
Thermoregulation
Ventilation- CPAP, High flow O2 via nasal cannula
Avoid formula milk (NEC)- use umbilical vein
Sufactant via ET tube
11
Q
How are premature neonates fed?
A
No suck reflex
Central access and parenteral nutrition via the umbilical vein
Then Train parents to give NG feeds +/- Discharge
12
Q
Meconium stained liquor in the presence of what would make you call the neonatal team?
A
Haemodynamic stress RR>60, HR>160<1000
Grunting
T>38
<95% sats
13
Q
Process of newborn resus
A
1) Dry baby and start clock
2) Assess Tone, HR, Breathing
3) Airway opening and 5 rescue breaths 250ml bag
4) HR increase? If not check chest movements and repeat inflation breaths
5) HR<60 + Chest movements= CRP! 3:1
14
Q
Describe the chest compressions used in neonatal resus
A
3:1 Compressions: Breaths
1/3rd Depth
~100/min
Reassess every 30s
15
Q
Describe the inflation breaths used in neonatal resus
A
2-3s per breath
Make sure chest is moving
16
Q
Position of the head when doing airway manoeuvres in neonates
A
Neutral
17
Q
When do you assess APGAR score?
A
1, 5 & 10 minutes
18
Q
What constitutes a good (score of 2) grimace on APGAR?
A
Sneezes, Coughs, Pulls away
19
Q
When is Neonatal jaundice always pathological?
A
<24 hours
20
Q
Physiological jaundice
A
> 24 hours
Resolution in 14 days
Usually beacuase of immature hepatic function and poor feeding
21
Q
Causes of unconjugated jaundice with onset <24 hours
A
Sepsis Haemolytic disease TORCHES G6PD Spherocytosis
22
Q
Key Investigations for neonatal jaundice
A
Urgent serum bilirubin needed with 2 hours Blood film Coombs Haematocrit Blood groups \+ LFTs, FBC, TFTs
23
Q
Causes of Unconjugated jaundice onset >24 hours
A
Sepsis Haemolytic disease TORCHES Metabolic disorders HYPOthyroidism Breast milk Physiological
24
Q
What hepatic abnormality does conjugated jaundice usually indicate?
A
POST-HEPATIC PROBLEM
25
Key distinguishing features of conjugated jaundice
Typically post-hepatic
Dark Urine, Pale Stools (NO BILE)
Pruritis
26
What is the direct bilirubin level in conjugated jaundice
>25 umol/l
27
Causes of conjugated jaundice
```
Liver disease
Biliary atresia
Choledochal cyst
CF
Hepatitis
Iatrogenic
Metabolic
```
28
Coomb's test
+ve= Haemolytic disease of the newborn
| Demonstrates a reaction between mum and foetus
29
Biliary atresia
Conjugated jaundice
No biliary tree
INR is very high
No vitamin ADEK absoprtion
30
LFTs in Biliary atresia
Transaminase rise
| GGT most raised
31
Definitive Treatment for biliary atresia
Kasai's Hepatoportoenterostomy
32
Management of jaundice
Continue to breast feed, adequate hydration
Treat cause
Phototherapy?
Blood Transfusion?
33
Phototherapy treatment for jaundice
Makes bilirubin water soluble for excretion
> Blue line= Treat
Can repeat bilirubin test in 24 hours if within 50 of blue line and no RF
Stop when >50 micromol/litre below blue line
34
How do you measure bilirubin?
1st 24 hours/<35 wks gestation= Serum
>35 weeks/>24 hours= Transcutaneous bilirubinometer (if >250 micromol/l then also do serum)
35
Indications for intensifying Jaundice Phototherapy
No fall after 6 hours
rising >8.5 micromol/l per hour
Pre-exchange
36
Monitoring of bilirubin during jaundice phototherapy
4-6 hours post start
| every 6 hours later
37
Indications for an exchange blood transfusion in neonatal jaundice
Serum Bili >450 (> Red line)
| If only just > Red try Phototherapy 1st?
38
Kernicterus
Unconjugated bilirubin in hyperbilirubinaemia collects in the basal ganglia
= Dystonic Cerebral palsy
39
Prolonged jaundice
> 2 weeks in term
>3 weeks in preterm
LIKELY BREASTFEEDING but ?Biliary atresia
40
What is raised serum bilirubin?
>100 mmol/L
41
Signs of HIE
Low APGAR (<5 at 5 mins)
Acidosis PH <7
Early encephalopathy
Bradycardia, Reduced Tone, Respiratory depression
42
Management of HIE
RESUS
IV/Art line
Avoid hyperthermia aim: 33-36 using cooling mat
Room air
? Fluid resus ? Omit milk and feed slowly
Treat seizures
43
Classification of birthmarks
Vascular (Red, Pink, Purple)
| Pigmented
44
Salmon Patch/Stork mark
Most common vascular birthmark
Crying= Inc with blood= More noticeable
Eyelids, neck, forehead
Flat red/pink
45
Port Wine stain
Rare, Mostly permanent
Glaucoma if on eyelid
Hormone sensitive
Darker than Stork marks
46
What syndrome are port wine stains associated with?
Sturge-Weber Syndrome (SEIZURES + LEARNING DISABILITY)
47
Treatment of port wine stain
Laser
48
Strawberry mark
Infantile Hemangioma
Red and Raised
May initially increase in size
?Topical propanolol
49
Cafe-Au-Lait Spotys
Coffee coloured spots
| GP review ?NFT1 if >6 by age 5
50
Mongolian Blue Spots
Look Like Bruises
Usually Buttocks
Gone by age 4
51
Cephalohaematoma
Subperiostal bleed + Suture lines limit the swelling
Complicated delivery increases risk
JAUNDICE IS A COMPLICATION
52
Pathophysiology of Rhesus Haemolytic disease of the newborn
F Rh +ve M Rh-ve
Foetal blood haemorrhages into maternal circulation
Anti-IGD production
Foetal RBC haemolysis at increasing severity with subsequent pregnancy
53
Post-natal presentation of Rhesus Haemolytic disease
Hyperbilirubinaemia + Jaundice
Hepatosplenomegaly
Hydrops fetalis
Throbocytopenia and Leucopenia
54
Hydrops fetalis
Fluid accumulation in abnormal compartments
| Ascites/Pericardial effusion/Pleural effusion/Skin Oedema
55
Neonatal blood sample results in Rhesus haemolytic disease
Low Hb, High reticulocytes, Low platelts, High serum bilirubin
56
Management principles in haemolytic disease of the newborn
```
Close supervision
O-ve ready
Jaundice management as per
Blood transfusion if severe anaemia
Oral folic acid 250mcg/kg/day
Weekly Hb check
```
57
Sufactant production and deficiency
Usually produced by week 30 via T2 pneumocytes
Deficiency leads to alveolar collapse, Hypoxia and shunting (No V but Q there)
58
RDS presentation
Likely <32 weeks gestation
| Haemodynamic compromise, see-saw breathing, Grunting
59
RDS on CXR
Ground Glass Appearance
| Generalised atelectasis, Airbronchograms, Decreased lung volume
60
RDS on a blood gas
Respiratory acidosis on blood gases as no ventilation
61
Management of RDS
Position: PRONE
ET Sufactant (Curosurf/Survanta)
? Intubation ?CPAP via Nasal Cannula
Gentamicin and Penicillin and stop once congenital pneumonia is excluded
62
Complications of RDS
Major one is Bronchopulmonary dysplasia caused by mechanical ventilation
O2 needed >28 days of life
Steroids and diuretics needed
63
Prevention of RDS
Betamethasone/Dexamathasone 2X12 hourly
| Give to Mother 1-7/7 before birth
64
What is generally thought to indicate fetal viability?
24+ weeks
| 500G
65
Respiratory complications of prematurity
RDS
Chronic lung disease
Apnoea of prematurity
66
CNS complications of prematurity
IVH
Periventricular leukomalacia
Retinopathy
67
Diagnosis of IVH
Cranial USS
| Head circumference is also a good indication
68
Perventricular leukomalacia
White matter softens near ventricles
69
Retinopathy screening
From 28 days
| Or if <1.5kg
70
Pathphysiology and treatment of retinopathy of prematurity
Abnormal vessel growth ?Proliferated by oxygen
| Treat with laser therapy
71
Presentation of NEC
Distension, Billious vomiting, Bradycardia, Erythema, Apnoea
PR bleeding
PREMATURE BABY
72
AXR signs of NEC
```
Intramural gas
Gas in the portal tree
Asymmetrical dilated bowel loops
Pneumoperitoneum
Air inside and outside bowel wall (Rigler's)
```
73
Treatment of NEC
Broad Abx- Cef + Vanco
Stop feeds 7-10 days
IV fluids and parenteral nutrition (?TPN ?NG)
Laparotomy if deterioration as could be perforation
74
TORCHeS
Congential infections
| Toxoplasmosis, Rubella, CMV, Herpes, Syphilis
75
Post-natal management of a premature baby
```
ICU
Delay cord clamping
Resp support
BREAST FEED or Parenteral via umbilical vein
Minimal handling
Keep warm
Monitoring
Benpen or Gent
```
76
IUGR/Small baby definition
Birth Wx <10th centile
77
Causes of small baby
```
Constitutional
Multiple pregnancy
Maternal abuse- smoking etc
Genetics
Placental insufficiency
TORCHeS
```
78
Symmetrical vs Asymmetrical growth restriction
Symmetrical- Early insult e.g infection ? Constitutional
Asymmetrical- Late insult like placental problem, head sparing effect
79
Complications of small baby/IUGR
```
Foetal death
Hypothermia/Glycaemia
NEC
Polycythaemia
BM/Hepatic compromise- Low platelets and coagulopathy
Retinopathy
Meconium aspiration
```
80
Discharge of Small baby/IUGR
Feeding well, Weight increasing, no thermoregulatory support room temp=Body temp
Mum capable
81
When would you admit a small baby to a neonatal ward
Consider if Wx<1800g
82
Talipes Equinovarus 'Club foot'
Inversion
Adduction of forefoot
Plantarflexion deformity
83
Talipes treatment
Ponseti method- Gradual correction via plaster cast
?Surgery if >2 years old
84
Live vaccines
MMR, TB. FLU, Rotavirus, Chicken Pox, Sabine vaccine
85
CI to vaccines
When do you delay
Hx Anaphylactic reaction (Consider in controlled environment), Egg allergy
Live: Immunocompromised
Delay: Evolving neurology, Acutely unwell, IG given recently
86
Inactivated/Killed vaccines
```
Polio
Pertussis
HiB
Men C/B
Tetanus/Diphtheria (Toxoid)
```
87
Administration of a vaccine
Thigh (Infant)
Deltoid (Older)
IM (SC if bleeding disorder)
88
Vaccines in preterm infants
Give at chronological age
89
6 in 1 vaccine
Diptheria,Tetanus, Whooping, Polio, HiB, Hep B
2/12, 3/12, 4/12
90
When is MMR given
12-13 months, 3-4 years
91
Men ACWY
14 Years
Students
Foreign travel
92
4 in 1 pre-school booster
3-4 years
| Diphtheria, Tetanus, Whooping, Polio
93
PCV
Pneumococcal vaccine
| 3 months
94
BCG
TB vaccine
Live attenuated
If lived in a high risk country or parents from a high risk region
At birth
95
Side effects of the MMR
5-10/7 later a rash/fever
| Mild mumps 2 weeks later
96
3 in 1 teenage boost
Tetanus, Diphtheria, Polio
97
HPV vaccination
Boys and Girls
| 12-13 years
98
Annual flu vaccine
2-8 years
| Likely Nasal Spray
99
Oral Rotavirus vaccine
2 months, 3 months
100
Men B and Men C vaccinations
Men B- 2 months, 4 months, 12 months
Men C given with HiB at 12-13 months
101
Complications of Measles, Mumps and Rubella
Measles- Encephalitis, SSP, Pneumonia
Mumps- Infertility, Deafness, Meningitis, Encephalitis
Rubella- Dangerous in pregnancy
102
Which type of vaccines classically require multiple boosters
Inactivated toxin vaccines
| Tetanus, Diphtheria
103
Bacterial Neonatal meningitis causes
GBS
Listeria
E.Coli
104
Bacterial Infant meningitis causes
S.Pneum
N.Meningitidis,
HiB
105
Neonatal/Infant meningitis symptoms
```
Non-specific
Fever without focus
Poor feeding
Irritable
Cold peripheries
Seizures
Respiratory distress
```
106
Signs of meningitis
```
Decreased consciousness
Bulging fontanelle
Bulging fontanelle
Cushing's- HTN, Bradycardia, Low RR
Brudzinski's and Kernig's
```
107
Brudszinski's and Kernig's signs
Meningeal irritation
Brudzinski's- Pain on knee extension when hip flexed
Kernig's- Flexion of the head= Hip flexion (90% sensitivity!)
108
Signs of raised ICP
```
Headache, Vomiting, Papilloedema
Mental state change, Pupilliary irregularity
Hemiparesis
HTN
Squint
```
109
CI to a Lumbar puncture
Focal neurology, seizures
Shock
Abnormal clotting
Meningococcal septicaemia
110
Bacterial infection as indicated by LP
Cloudy CSF
High Neutrophils>Lymphocytes
High Protein
Low Glucose
111
Viral infection as indicated by LP
Clear/Cloudy CSF
Lymphocytosis
Normal glucose
Potentially normal protein
112
TB as indicated by LP
Slightly cloudy CSF
| High lymphocytes, V high protein, Low Glucose
113
Empirical management of Bacterial meningitis
IV Cefotaxime and Amoxicillin if <3/12
IV Ceftriaxone
Alter once organism known
114
Abx of choice of Meningococcal/Pneumococcal/Hi meningitis
Cefotaxime
115
Delayed complications of Meningitis
Hearing loss
Focal paralysis
Seizures
CP
116
Hearing test post-meningitis
6/52 post-discharge
117
Meningitis chemoprophylaxis
Same house, > 5 hours per day in room with meningococcal child
Give Cipro or Rifampicin
118
Meningococcal septicaemia
N.Meningitidis G-ve diplococci inducing bacteraemia and vasculitis
Fever, Mottled, pain, Cold peripheries, breathing problems
Later have non-blanching rash +/- Confusion
119
Treatment of Meningococcal Septicaemia
IM BenzylPenicillin or IV Cefotaxmine
120
Meningitis vs Meningococcal septicaemia
Speticaemia is likely to induce a rapidly spreading purpuric rash
Purpura are 2-10mm
121
Neonatal sepsis treatment- Abx
<1 month- Try Benzyl and Gent
1- 3 months- Cefotaxime
122
Petechial spot vs Purpura vs Ecchymosis
Petechial spots are <2mm
Purpura are 2-10mm
Ecchymosis >10mm
123
What is purpura
Non-blanching red/purple discolouration of the skin, 2-10mm
124
Causes of purpura
```
Meningococcal sepsis
Thrombocytopenia
HSP
Viral infection
Vasomotor straining
Trauma
VWD
```
125
Presentation of sepsis in children
```
Tachypnoea/Cardia
Bradycardia
Dry nappies
Mottled/Ashen appearance
Non-blanching purpuric rash
Low sats
```
126
Neonatal conjunctivitis
Sticky eyes D3-D4, non-infective
Gonococcal- 1st 48 hours, purulent discharge
Chlamydial- 7-10 days LATER
127
Infective vs Allergic conjunctivitis
Latter has bilateral chemosis and swelling
Former may have purulent discharge and eyes stuck together
128
Common causes of infective conjunctivitis
Adenovirus
G+ve Cocci
H.Influ
129
Varicella Zoster Virus
Chicken pox
Highly infectious
Spread via respiratory droplets a direct contact with lesions
130
Describe the rash in chicken pox
```
Starts on head/trunk then spreads
Red macule (Flat) -> Papule-> Vesicle-> Pustule -> Crusting
```
131
When is Chicken Pox infectious
4 days before rash till around 5 days after
Once crusted over it is no longer infectious
132
How can you manage chicken pox related itching
Calamine lotion, chlorphenamine (>1 yr), cooling baths, short nails
133
School exclusion in chicken pox
5 days post-skin eruption
134
When in chicken pox is varicella IG given
Immunocompromised- prophylaxis
Baby born with chicken pox within 7/7 of mother contracting VZV
135
When would you consider Aciclovir for chicken pox
Severe systemic infection, pneumonia, Encephalitis, Immunosuppressed, Babies
136
Complications of VZV
Secondary bacterial infection
Encephalitis
Purpura fulminans- Disseminated Haemorrhagic chicken pox
Pneumonitis
137
VZV vs HZV
VZV is chicken pox
HZV is shingles in later life when latent VZV is reactivated
138
Triad of symptoms classically seen in Infectious Mononucleosis/Glandular fever
Exudative pharyngitis (Sore throat)
Generalised tender lympthadenopathy
Pyrexia
139
Lympthadenopathy in EBV vs tonsilitis
Tonsilitis only enlarges the upper anterior cervical chain
In EBV it is generalised and tender
140
Investigations to confirm Dx of Infectious Mononucleosis
>10% Atypical lymphocytes on blood film + +ve EBV serology + Number of lymphocytes increases to 85% of leucocytes/RBC
Mono Spot test (Low sensitivity)
Raised LFTs, IgM,IgG
141
Complications associated with Infectious Mononucleosis
Hepatitis and Splenomegaly
Aseptic Meningitis, Encephalitis, Guillan-Barre Syndrome
Orchitis, ITP, Pneumonia
142
Presentation of Cow's Milk Protein Allergy
Presents in 3/12 of forumla feeding
Faltering growth
Multi-system involvement- GI, Dermatology, Resp wheeze
143
Management of Cow's Milk Protein Allergy
Hydrolysed formula and if that doesnt work try AA formula
Dietician support
Mum may have to not have Cow's milk
Majority resolve by 1 year
144
The Atopic March
General order in which atopic pathology develops
Eczema-> Food allergy -> Hayfever -> Asthma
145
Investigations for food allergy
Gold standard is a supervised challenge
Skin Prick test -> if not suitable then try Radioallergosorbent test (RAST/ELISA) which grades amount of IgE reacting
Skin patch testing for contact dermatitis
146
Presentation of allergy in a child
```
Diarrhoea +/- Blood/Mucus
Faltering growth
Skin changes- Erythema
Wheeze
Anaphylaxis
```
147
Clinical Diagnosis of Kawasaki Disease
Unexplained Fever > 5 days >38.5 degrees
+4/5 of:
- Non-purulent bilateral conjunctivitis
- Lips/Oral changes (E.G. Erythema)
- Extremity changes (E.G palmar erythema)
- Polymorphous Rash
- Cervical lymphadenopathy
148
Ages in which Kawasaki Disease is most commonly seen
<5 years
149
Management of Kawasaki Disease + Who must review these patients?
High dose IV Ig- 2g/kg over 12 hours
Aspirin- 30-50mg/kg/day QDS then low dose for 6 weeks
Cardiac review- ECHO!
150
Scarlet Fever vs Kawasaki Disease
In scarlet fever the rash is Blanching and Punctate
| There will also be exudative tonsillitis and respoonsiveness to penicillin V in scarlet fever
151
Key complications of Kawasaki disease
Coronary artery aneurysms
+ Coronary thrombosis, MI, Dysrhythmias, Reye Syndrome
152
What does Aspirin cause in children
Reye syndrome- Liver and Brain swelling-> Encephalitis
N.B. only used in Kawasaki's
153
What is the Measles virus
Morbillivirus
RNA Paramyoxovirus
Spread by respiratory droplets
154
When is measles infective
Infective 4/7 before rash onset till 4/7 after rash onset
155
Triad of Cs in Measles
Conjucntivitis
Cough
Coryzal
156
Mouth changes pathognomonic of measles
Koplik spots
| White spots opposite the molars
157
Describe the rash associated with measles
Maculopapular
FACE AND BEHIND EARS INITIALLY
then can spread
Become blotchy and confluent
158
Investigations for measles if doubt about diagnosis
```
Buccal swab
IgM levels
Blood film
LFTs
oral fluid test- measles RNA
```
159
What happens to WBC levels in measles
Leucopenia
| Lymphopenia
160
Complications of measles
ACUTE OTITIS MEDIA
+LRTI, Pneumonitis, Febrile convulsions, Encephalitis, SSP
161
What is Subacute Sclerosing Panencephalitis
Late complication of mealses 1-7 years post infection
| White matter damage- Fatal
162
Periorbital vs Oribital Cellulitis
Periorbital is less serious: Erythema/Oedema and tenderness around eye, can progress to orbital involvement
Orbital: Limited occular movement, Decreased acuity, Evolving occular proptosis
Both need IV Abx
163
Common causes of periorbital cellulitis
Staph A
| HiB
164
Which method of delivery has the lowest risk of vertical transmission of HIV
C-section
165
Triad seen in Foetal Rubella Syndrome
Cardiac changes
Cataracts
Deafness
166
Presentation of Rubella
Prodrome illness with mild fever
| Maculopapular rash starting on the face and spreading over the whole body lasting up to 5 days +/- Lymphadenopathy
167
What Virus causes Rubella
Togavirus
168
When is Rubella infectious?
7 days before symptoms
| 4 days after onset of rash
169
Normal Temperature
| Low grade and High grade fever
Normal= 36.5-37.5
Low grade= >37.5
High grade= >38.5
170
3 important vital sign measurements in a child with fever
HR
RR
CRT
171
Red flag signs in a child with fever
```
Pale/Mottled/Ashen/Blue
Weak high pitched cry
Not responding/Cannot stay awake
Grunting
RR>60
Chest indrawing
< 3 months and Temp >38
Non-blanching rash
Neck stiffness
Focal neurology/seizures/Status
Bulging fontanelle
Decreased skin turgor
```
172
Features indicating you should have a low threshold for a septic screen in a child with fever
```
No localising features
< 3 months
Systemically unwell
Features of a potentially serious infection
Behavioural change
Predisposition to infection
```
173
Features indicating you should have a high threshold for a septic screen in a child with fever
Older child
Not systemically unwell
Localising features
174
What is the septic screen
```
Blood cultures
Urine dipstick and culture
FBC, CRP, U&Es
LP
CXR
+/- Stool culture if bloody diarrhoea
```
175
Non-infectious causes of fever
```
Thyrotoxicosis/Hyperthyroidism
JIA
IBD
Dehydration
Malignancy
```
176
Amber flags in a child with fever
```
Pallor reported by carer
Wakes with prolonged stimulation
Temo >39 in 3-6/12
Low O2 sats, High RR but <60
Nasal flaring
Crackles
Tachycardia
Decreased UO
Poor feeding
Rigors
Not weight bearing
```
177
How do you manage a Child with fever + Red flag signs
Urgent referral to paediatrics
| Septic screen
178
Clinical dehydration vs Shock
Both show inc HR, RR, Decreased turgor and decreased UO
In shock there is pale cole extremities, prolonged CRT, and decreased consciousness
Hypotension and bradycardia will then develop as they decompensate
179
Causes of Iron deficiency anaemia in Children
Nutrition- Delayed introduction of iron rich foods post breastfeeding, Cow's milk enteropathy, Diets
Adolescent females- Menstruation, Growth spurts
Obstetrics- Preterm, LBW, Multiple pregnancy
Others- Malabsorption, Rarely blood loss, parasites
180
What does Iron deficiency anaemia present as on a FBC/Haematinics
```
MICROCYTIC HYPOCHROMIC ANAEMIA
Low Hb
Low MCV
Low MCH
Low MCHC
Low Ferritin
```
181
How do you prevent Fe deficiency anaemia in high risk groups
Iron supplements for preterm infants
Iron containing diet- fortified milk formula, meat, green veg, beans, Vit C
Avoid prolonged cow's milk consumption
182
Management of Iron deficiency Anaemia
5mg/kg oral ferrous sakt in 2-3 divided doses
Continue for 3/12 after Hb has normalised to increase stores
Transfusion if no response
183
Max dose of oral iron a day for children
200mg/day
184
Most common central paediatric malignancy and paediatric brain tumour
Malignancy= Medulloblastoma
| Paed brain tumour= Pilocytic astrocytoma
185
Low grade astrocytomas
Gliomas
Mostly Pilocytic astrocytomas
NF1 can lead to low grade gliomas on the optic pathway
186
High grade gliomas
Difficult to manage as complete resection is hard
Mostly supratentorial
Diffuse brainstem gliomas are inoperable
187
Primitive Neuroectodermal tumours (PNET)
This category of tumours comprises the most common cause of malignant brain tumours in childhood
Medulloblastoma is in this category- Cerebellum and aggressive
Peak 2-6 years
20% have mets to the spine at Diagnosis
188
What sign do Ependyomas classically cause?
Obstructive Hydrocephalus as these are periventricular tumours
189
Examples of CNS Germ cell tumours
Teratoma, Germinoma
| Secreting tumours so look for markers
190
Common presentation of cranipharyngioma
Visual field loss and pituitary dysfunction
| B/C NEAR PITUITARY GLAND as squamous remenant of Rathke's Pouch that doesn't mature into the anterior pituitary gland
191
Presentation of retinoblastoma
```
Absent or abnormal light reflex
Leukocoria
Squint
Visual disturbance
Uni- or bilateral
```
192
Epidemiology of retinoblastoma
18 months is most common age at presentation
Unilateral- 2-3 years
Bilateral- 0-12 years
BUT 90% 5 year survival with treatment
193
Aetiology of retinoblastoma
Loss of TSG on chromosome 13
| ~10% Hereditary
194
What is Haemophilia?
X-Linked recessive disease
A- F8 production is defective
B- F9 production is defective (Less common)
195
Presentation of haemophilia
Haemathorases, Easy brusing, Epistaxis, IM Intracranial
```
If severe (<1% activity) then there will be spontaneous bleeding
In moderate (1-5% activity) bleeding is secondary to trauma
If mild they will rarely bleed (5+% activity)
```
196
Haemathorases
Bleeding into joint space- can be a sign of haemophilia
Swollen, Painful, Tender, Warm, Limited movement, cannot weight bear
197
APTT and PT results in haemophilia
APTT is increased
PT is normal as this looks at F7
198
What method of injection is best in haemophiliacs
IV, SC
IM can cause bleeding +/- Compartment syndrome and nerve compression
199
Haemophilia and surgery
Prophylactic DDAVP/desmopressin + Factor + Aminocaproic acid (Anti-fibrinolytic)
May need haematology input
200
Haemophilia prophylaxis and on demand drugs if severe
A= Octocog alpha (F8) every 48 hours +/- Desmopressin (stimulates VWF production)
Nonacog alpha used in B
201
Triad of symptoms in HSP
Buttocks +/- Lower leg papular purpuric rash
Non-destructive polyarthritis
GI involvement- Colicky abdo pain +/- Haematemesis
202
Why is a dipstick a key investigation for HSP
HSP can lead to Glomerulonephritis
| The dipstick can detect haematuria and proteinuria
203
What is HSP
IgA immune complex mediated small vessel vasculitis commoly seen post-infection
Especially in pre-pubertal boys
204
Management of HSP
NSAIDS for polyarthritis
IV steroids if abdo pain and N&V
?Renal input
Most resolve in 6 weeks
205
Most common childhood malignancy
Leukaemia
| Specifically Acute lymphoid leukaemia
206
Pathophysiology of ALL vs AML
ALL- Malignant proliferation of B/T cell precursors in the bone marrow
AML- Cells of granulocytic/Monocytic lineage
207
Age at which Leukaemia commonly presents
2-6 years
208
Symptoms suggestive of leukaemia
```
Malaise, Anorexia
Anaemia= Lethargy, Pallor
Neutropenia= Frequent, severe infections
Bruising
Lympthadenopathy
Organomegaly
```
209
Blood results in Leukaemia
Anaemia
Thrombocytopenia
Neutropenia
PT increased
210
Poor prognostic factors for ALL
```
Age<2 years or >10 years
WBC>20x10^9/L
Non-caucasian
Male
T or B cell surface markers
```
211
ALL with orthopaedic involvement and on an X-ray
Patient may present with limb pain +/- Fractures
Radiological lucency (Lower density than surrounding tissue)
212
Presentation of lymphoma
Progressive painless lymph node enlargement
Mediastinal/Intrathoracic/Cervical mass
B symptoms- Fever, Weight loss, Night sweats
213
Epidemiology of Hodgkin's vs Non-Hodgkin's lymphoma
Hodgkin's- Adolescents and young adults
| Non-Hodgkin's- Young children
214
EBV is associated with increased risk of what lymphoma?
Hodgkin's
215
Reed-Sternberg cells
Hodgkin's lymphoma
216
Investigations in Lymphoma
CT scans
FDG PET scan (Tracer)
BM Aspiration
Isotope bone scan especially if B symptoms
217
Ann Arbor staging
Hodgkin's lymphoma
218
Genetics of Sickle cell anaemia
Autosomal recessive
Inherited defect in HBA Beta chain
Increased in Carribean, African, Middle East, Indian
219
Pathophysiology of Sickle cell anaemia
Long inflexible chains of Deoxy-HbS polymer
Vaso-Occlusion and haemolysis leads to increased viscosity and decreased blood flow
Therefore: Tissue Infarction and Haemolytic anaemia
220
Onset and course of SC anaemia
Fluctuates between good health and crises
| Homozygotes onset in 4/12 after HbS takes over from Foetal Hb
221
Features of SC anaemia
Dactylitis, HYPOsplenism, Vaso-occlusive crisis in long bones, Avascula rnecrosis, Priapism, Nephropathy, retinopathy and decreased growth
222
Key features of a SC crisis
```
Pain B/C vaso-occlusion
Lung collapse (Shadowing on X ray)
Severe anaemia as BM fails
Life threatening hypovolaemia as the spleen suddenly increases in size
Cerebrovascular occulsion... Stroke
Priapism
```
223
Acute management of a SC crisis
Analgesia
Antihistamine (Histamine increases SC adherence)
O2 and fluids (?150% of normal maintenance)
Transfusion if aplastic crisis or sequestration
?Abx
224
Maintenance therapy in SC anaemia
Hydroxycarbamide to decreases crisis frequency
L-Glutamine to decrease pain
Lifetime penicillin prophylaxis + Vaccinations
Daily oral folic acid and high cell turnover
Repeated blood transfusion
BM transplant is curative
225
What can precipitate a SC crisis
Hypoxia
Cold
Dehydration
Infection- Parvovirus B19
226
What is Neuroblastoma? Peak Onset?
Solid malignant embryonal tumour arising from the sympathetic nervous system
Children <5 years
227
Features of neuroblastoma
```
Abdominal pain, distension and mass
Panda eyes (Perioribital bruising)
Horner's syndrome
Airway or Vene cava compression
Pallor and Wx loss
```
228
Thalassemia pathophysiology
Defect in the synthesis of >1 globin chains
Either the alpha or Beta chain of the Hb molecule
Unlike SC anaemia these chains are missing
229
Alpha vs Beta Thalassemia
Beta is the most common
Alpha is mostly asymptomatic, if 2-3 chains affected symptoms show, 4 chains usually means death in utero
Variable severity anaemia
230
Genetics of Thalassemia
Autosomal Recessive
231
Beta Thalassemia signs/symptoms
```
Mild Hypochromic Microcytic Anaemia
Large head
Misaligned teeth
Abd distension
Failure to grow
Lethargy
Jaundice
Marked Hepatosplenomegaly in homozygotes
```
232
Diagnosis of Thalassemia
Hb Electrophoresis
In Beta there will be high HBA2 and HbF
Alpha usually has normal levels
233
Management of Thalassemia
Regular blood transfusions with chelating agent desferrioaxmine to prevent haemosiderosis
Folic acid
234
What is a Wilm's tumour?
Embryonal tumour of the kidney
Nephroblastoma
Classically a sproadic mutation
1/3rd involve WT1 on Chr 11
235
Peak age of onset for a Wilm's tumour?
2-5 years
| 90% <7 years
236
Presentation of a Wilm's tumour
Visible, painless, unilateral, abdominal mass
+/- Distension
+/- Haematuria
+/- HTN
237
What investigations must be done to exclude neuroblastoma in ?Nephroblastoma
Abdo USS + CT
CXR
Urine catecholoamines
Also do FBC, Coag studies, Renal function
238
Treatment of a Wilm's tumour
Nephrectomy + Chemotherapy +/- RT
239
Cyanotic congential heart diseases
Tetralogy of Fallot
Transportation of the great arteries
Tricuspid atresia
Truncus arteriosus
240
Non-cyanotic congential heart diseases
Coarctation of the aorta
ASD/VSD/AVSD
Patent DA
241
What are the 7 S' of an innocent murmur
```
Soft
Systolic
Short
Sternal edge (L)
Symptomless
Sensitive to position change
Sweet in pitch
```
242
Still's murmur
Flow murmur across the aortic valve
Changes with position
Musical sound
243
Pulmonary flow murmur
Brief and high pitched
2nd LICS
Turbulent flow
244
Venous Hum
Heard below clavicles
| Continuous Hum caused by venous return
245
When listening to a heart murmur what do you assess?
Position
Character
Grade
246
Eisenmenger's syndrome
When any L to R shunt leads to RVH
| As a consequence the shunt is reversed to R to L
247
Where do the umbilical veins drain
IVC
248
What does the ductus arteriosus do?
Connects the pulmonary artery to the aorta
| Blood flows down from the pulmonary artery into the aorta
249
If a newborn becomes ill in the first few days of life what does it suggest about the congenital heart condition
That it is duct dependent
| Think Coarctation of the aorta or Transportation of the great arteries
250
What makes up the tetraology of Fallot
Overriding aorta
Large VSD
RVH
Stenotic pulmonary artery
251
Symptoms of ToF
Intermittent cyanosis from birth
Tet spells where they become hypercyanotic
Will not thrive
252
Murmur in ToF
Loud ejection systolic murmur B/C Stenotic pulmonary artery
253
Heart shape in ToF on a CXR
Boot shaped
254
Treatment of transportation of the great arteries
Prostaglandins to maintain the DA (Alprostadil)
Emergency atrial septostomy (ASD)
Atrial switch operation
255
Commonest heart defect in Down's syndrome
AVSD
256
Murmur in ASD/AVSD
Split S2 as venous return overloads pulmonary valve
| Soft ejection systolic murmur
257
Shape of the heart in Transportation of the Great arteries on CXR
Egg on its side
258
Commonest cardiac defect
VSD
| ToF is the commonest cyanotic defect
259
Murmur in VSD
Harsh Pansystolic
260
When does VSD usually present
4-6 weeks as L to R shunt gets bigger
SoB on feeding
Recurrent infections
?HF
261
Diagnosis of Congenital Heart Disease
Echocardiogram
262
What maintains the patency of the ductus arteriosus
Prostaglandins
263
What can precipitate a patent Ductus Arteriosus
Valproate, Congenital Rubella syndrome, Preterm, Hypoxia
264
Murmur in patent Ductus Arteriosus
Machinery Hum- Continuous
Tricuspid area
+/- Subclavicular thrill
265
What infection does congential heart disease increase your risk of
Endocarditis
266
Treatment of Patent Ductus Arteriosus
Ibuprofen
Diuretics for HF
+/- Surgery
267
How does Ductus Arteriosus closure impact Coarctation of the aorta
Leads to arterial constriction where some of this ductal tissue has extended into the aorta thereby increasing the obstruction
268
Presentation of Coarctation of the Aorta
Radiofemoral delay (Subclavian artery not affected by the narrowing)
Discrepancy between pre- and post-ductal sats
Ejection systolic murmur
Shock and metabolic acidosis
269
Management of Coarctation of the Aorta
IV prostaglandins to maintain the patency of the Ductus arteriosus
Diuretics if HF
Stent
Surgical correction post-diagnosis by echo
270
Abx of choice for meningococcal sepsis
Cefotaxime
271
Scarlet fever
Sandpaper rash- Fine punctate erythema on head and trunk but sparing palms and soles
Palatal petechiae- RED THROAT
Strawberry tongue that can intially be white
Caused by GAS
272
Scarlet fever management
PO Penicillin V
| Stay home from school for 24 hours post-starting Abx
273
Urticaria
Raised red rash
Itchy
Wheals
274
CI to lumbar puncture
```
Local infection
Unconsciousness/Decreasing GCS
Meningococcal septicaemia likely
Bleeding disorder
Abnormal posture
Respiratory abnormality- Hyperventilation, Cheyne stokes
Seizure >10 mins, focal seizure
Pupils abnormal
Raised ICP
Focal neurology
Shock
```
275
Impact of positive pressure ventilation on a preterm baby
Can lead to pulmonary fibrosis and bronchopulmonary dysplasia so try and avoid
276
How do baby radiographs differ from adults?
When supine the clavicles have a lordotic projection
Cardiothoracic ratio <0.6
Wide mediastinum because of big thymus
Very big gastric bubble and kinked trachea B/C crying
277
Normal vital sign values for a child <1 year
HR 110-160bpm
RR 30-40
Temp 36.6-37.5
SBP- 70-90mmHg
