Paeds Flashcards
(239 cards)
1
Q
What MSK problems may cause pain without swelling?
A
Hypermobile joints
Perthes diseases
Metabolic - hypothyroidism
Tumour - benign or malignant
2
Q
Define juvenile idiopathic arthritis.
A
Joint inflammation in someone under 16 years for over 6 weeks (other causes excluded)
3
Q
How is JIA classified?
A
Number of joints affected in the first 6 months
4
Q
What are the classifications of JIA? (6)
A
Oligoarticular Polyarticular RF -ve Polyarticular RF +ve Systemic onset JIA Psoriatic Enthesitis related arthritis
5
Q
What is the most common classification of JIA and describe it.
A
Oligoarticular (50%) 1-4 joints affected in first 6 months 70% ANA +ve Ankle/knee Swelling, stiffness, reduced ROM, little pain
6
Q
Describe polyarticular RF -ve JIA.
A
5+ joints
Asymmetrical/symmetrical
Stiffness, little swelling
Destructive
7
Q
Describe polyarticular RF +ve JIA.
A
5+ joints RF seen on 2 occasions Symmetrical involvement of hand/wrist Possible rheumatoid nodules Systemic features - fever, HSmegaly, serositis, pericardial effusion
8
Q
Describe systemic onset JIA.
A
Arthritis with fever every day for >2 weeks 1 of: Rash Lymphedema HSM Macrophage activation syndrome (MAS)
9
Q
Describe psoriatic arthritis.
A
Arthritis + psoriasis or Arthritis + 2 of: Dactylitis Nail pitting Psoriasis in first degree relative
10
Q
Describe ERA
A
Arthritis + enthesitis or Arthritis + 2 of: Hx SIJ tenderness HLAB27 Onset over 6 years of age in males Acute anterior uveitis Reiter syndrome
11
Q
What complications can occur with JIA?
A
Chronic anterior uvetis Flexion contracture Growth failure Anaemia of chronic disease Delayed puberty Osteoporosis
12
Q
How is JIA diagnosed?
A
Clinically
13
Q
What may been seen on FBC of a patient with JIA?
A
Normocytic anaemia
Normal or raised WCC
Raised platelets
14
Q
What blood tests can be done for JIA?
A
ANA
RF
HLAB27
15
Q
What investigations may be done for JIA?
A
MSK examination X ray Ophthalmology review USS MRI
16
Q
What is the non-medical management for JIA?
A
Physiotherapy
Hydrotherapy
Physical activity
17
Q
What is the pharmacological treatment for JIA?
A
NSAIDs and analgesia
1st line - intra articular steroid injections
DMARDS - methotrexate, sulfasalazine
Biologics - rituximab, infliximab, tocilizumab
18
Q
What does tocilizumab target?
A
Interleukin 6
19
Q
What does infliximab target?
A
TNF-alpha
20
Q
What does rituximab target?
A
CD20 cells
21
Q
Why should systemic steroids be avoided for JIA?
A
Risk of osteoporosis
Growth supression
22
Q
What is osteomyelitis?
A
Long bone infection affecting the metaphysis
23
Q
How does osteomyelitis occur?
A
Haematogenous spread
Adjacent site e.g. cellulitis
Penetrating trauma
24
Q
Where are the most common sites for osteomyelitis?
A
Femur
Proximal tibia
25
How old do children with osteomyelitis tend to be?
< 2 years
26
What organism cause osteomyelitis in an otherwise well child?
Staph aureus
Group A beta haemolytic strep
H. influenzae (if not vaccinated)
27
What is the causative organism of osteomyelitis in an immunocompromised child?
TB
28
What is the clinical presentation of osteomyelitis?
Severe pain
Immobile limb, severe pain on movement
Inflammatory signs over the affected area
Sterile effusion in adjacent joints
29
What can occur as a complication of osteomyelitis?
Septic arthritis
30
What would an X-ray of osteomyelitis show?
Initially normal
| After 7-10 days, shows new subperiosteal bone formation
31
What may be seen on FBC of a patient with osteomyelitis?
Raised WCC, ESR and CRP
32
How would you identify osteomyelitis infection?
MRI
33
What is the treatment of osteomyelitis?
Cefuroxime over 3 months (6 weeks minimum)
| Initially IV then switch to oral when CRP < 10, if tolerated, walking around or no collection
34
What abx would be given if blood culture showed staph aureus infection?
Flucloxacillin
35
What is septic arthritis?
Infection of the joint space.
36
Why is SA considered an emergency?
Can lead to bone destruction in < 24 hours.
37
When should SA always be considered?
In the limping child.
38
What is the peak incidence of SA?
< 2 years
39
How can a child get SA? (4)
Haematogenous spread Puncture wound
Infected skin lesion (chickenpox)
Spread from osteomyelitis (especially in hip)
40
What is the causative organism of SA in neonates?
Group B step
41
What is the causative organism of SA in adolescents?
Neisseria gonorrhoeae
42
What is also seen in SA caused by Neisseria gonorrhoeae?
Migratory polyarthralgia
Multiple joint involvement
Small red papules
43
What is the CP of SA?
Acute febrile child
| Erythematous, warm, acute tender joint with reduced ROM
44
Why is diagnosis of SA of the hip difficult?
Due to large amounts of SC fat.
45
What criteria is used to diagnose SA?
Kocher criteria
46
What are the points used in criteria to diagnose SA?
WCC > 12
ESR > 40
Fever > 38.5
Inability to weight bear
47
What is the gold standard investigation for SA?
USS guided joint aspiration and microscopy
48
What is the treatment for SA?
Surgical drainage followed by IV abx, usually for 6 weeks.
49
When might surgical treatment not be indicated in SA?
Neisseria gonorrhoeae infection.
50
What complications can occur with SA?
```
Femoral head destruction
Deformity
Joint contracture
Limb-length discrepancy
Gait abnormality
```
51
What is transient synovitis?
Inflammation of the synovium causing hip pain.
52
How does transient synovitis present?
Recent URTI
Acute/insidious onset of thigh/groin pain
Refusal to weight bear
53
What is seen on examination of transient synovitis?
Mild-moderate restriction of internal hip rotation, painless arc of motion.
54
How would you differentiate transient synovitis from SA of the hip?
FBC would be normal.
55
When should transient synovitis be diagnosed?
Only when everything else has been excluded.
56
What is the treatment for transient synovitis?
Supportive - rest and analgesia
| Self limiting within one week
57
What are risk factors for developmental dysplasia of the hip?
```
Being female
Breech presentation
Family history
First born children
Oligohydramnios
```
58
When is DDH usually noticed?
Newborn screening examination or 8 week examination
59
What two tests can be used to screen for DDH?
Barlow: attempts to posteriorly dislocate the articulated femoral head
Ortolani: attempts to relocate dislocated femoral head
60
What may be seen on inspection of DDH?
Asymmetry:
Gluteal or thigh skin folds
Limb length discrepancy
61
What may be observed on examination of DDH?
Limitation and asymmetry of hip abductors when flexed to 90 degrees
Benign hip clicks
62
How is DDH diagnosed?
Dynamic USS
| Pelvic X ray in older infants
63
What is the management of DDH?
Early diagnosis
| Appropriate alignment in first few months can mean DDH resolves spontaneously
64
What is osteogenesis imperfecta?
Inherited condition causing increased fragility of bone, lower density and joint laxity
65
What tissue does OI affect principally?
Those containing collagen type I - bones and teeth
66
Expect for bones and teeth, what other tissues can OI affect? (5)
Sclerae, joints, tendons, heart valves, skin
67
What is the inheritance pattern of OI?
Autosomal dominant.
68
Mutations in what genes caused OI?
COL1A1 and COL1A2
69
What can OI be mistaken for and why?
Child abuse and NAI - may be inconsistent history of injury frequency and severity.
70
How many presentations of OI are there?
Types 1-4.
71
What is the most common presentation of OI?
```
Type 1:
AD
Blue sclera
Hearing loss in 50%
Fractures before puberty
Normal life expectancy
```
72
What is the lethal perinatal form of OI?
```
Type 2:
AR
Many fractures
Blue sclera
Dwarfism
```
73
Describe osteogenesis imperfecta type 3:
```
AR
Fractures at birth
Progressive spine and limb deformity
Blue sclera
Dentinogenesis imperfecta
```
74
Describe osteogenesis imperfecta type 4:
Autosomal dominant
Fragile bones
White sclera after infancy
75
How is OI detected?
Antenatal diagnosis by USS
76
What is seen on X-ray of OI?
Low bone density
| Bowing of long bones
77
What does histology of OI bone show?
Immature, unorganised bone, abnormal cortex
78
How is OI managed?
Prevent injury, physiotherapy, rehabilitation, bracing
79
Medical management of OI? What effects does it have on the patients life?
Alendronate - reduces fracture frequency, reduces pain, increased mobility, no adverse effects on growth
80
When may surgery be indicated for OI?
To correct deformities - osteotomies, intramedullary rods.
81
What is Perthe's disease?
Idiopathic avascular necrosis of the proximal femoral epiphysis in children.
82
Who does Perthe's disease affect?
5-10 year olds, males > females
83
What is the pathophysiology behind Perthe's disease?
Osteonecrosis occurring secondary to disruption of blood flow to femoral head.
Followed by revascularisation, subsequent resorption and later collapse
84
Over what time scale does Perthe's disease occur?
Years
85
Name 4 symptoms of Perthe's disease.
1. Pain in hip/groin + referred pain to the knee.
2. Pain worse on activity, relieved by rest
3. Painful muscle spasms
4. Limp
86
What are the complications of Perthe's disease?
OA secondary to aspherical femoral head
| Premature fusion of growth plates
87
What may be seen on examination of a patient with Perthe's disease?
Limited abduction and internal rotation
88
What is seen on X-ray/MRI of Perthe's disease?
Space widening.
| Later signs - decreased femoral head size, patchy density, collapse and deformity.
89
What radiological classification is used for Perthe's disease?
Herring criteria.
90
What is the management of Perthe's disease based on prognosis?
```
High prognosis (< 6 years at onset) - observe
Low prognosis - surgery
```
91
How is prognosis determined for Perthe's disease?
Determined by risk of OA. Better in younger patients (< 6) as increased ability to remodel.
92
When is surgery indicated in Perthe's disease?
> 1/2 femoral head affected.
93
What are non-operative treatment options for Perthe's disease?
Limit activity, NSAIDs, physio, casting, bracing, protected weight bearing
94
What is SUFE?
Slipped upper femoral epiphysis
95
When does SUFE occur?
10-16 years during adolescent growth spurt
96
What is a major RF for SUFE?
Obesity.
97
What happens in SUFE?
Displacement of the growth plate with epiphysis slipping inferiorly and posteriorly
98
How does SUFE present?
```
Hip pain
Limping
External rotation of the hip
Flexion, abduction and internal rotation limited
90% able to weigh bear
```
99
Describe the onset of SUFE?
Acute following trauma or insidious
100
How is SUFE diagnosed?
Confirmed using X-ray (anterior and frog-leg lateral)
101
What is the treatment for SUFE?
Surgical fixation with pin to stabilise slippage and encourage physeal closing.
102
What conditions are associated with SUFE?
Hypothyroidism
Renal osteodystrophy
Growth hormone deficiency
Panhypopituitarism
103
What is Osgood Schlatter disease?
Self limiting disorder of the knee
| Osteochondritis of the patella tendon insertion at the knee
104
What thought to be the pathophysiology behind OSD?
Multiple small avulsion fractures from contraction of the quads at their insertion into the proximal tibial apophysis
105
When does OSD occur?
Adolescent growth spurt before tibial tuberosity has finished ossifying
Boys 12-15 years
Girls 8-12 years
106
What is the clinical presentation of OSD?
Gradual onset pain/swelling/tenderness over tibial tuberosity
Relieved by rest
Exacerbated by running/jumping - knee extension against resistance.
107
What is another name for OSD?
Tibial apophysitis.
108
What would a knee X-ray of OSD show?
Irregular apophysis
| Separation from tibial tuberosity
109
How is OSD treated? (4)
Rest from painful activities
Ice
Physio
Simple analgesia
110
Who does OSD affect?
Sporty teenagers
111
What is Kohler's disease?
Rare disorder affecting the navicular bone and causing foot pain in children (6-9 years).
112
What are the signs and symptoms of Kohler's disease?
Foot pain across tarsal region
| Limp
113
What does an X-ray of Kohler's disease show?
Dense, deformed bone
114
What is the treatment of Kohler's disease?
Rest
Cast in acute cases
Analgesia
115
What is the prognosis for Kohler's disease?
Good
| Few long term complications
116
What is discoid meniscus?
When meniscus is thicker than usual and often over or disc shaped
117
How does discoid meniscus present?
Can be asymptomatic
Pain, stiffness or swelling of the knee
'Popping' knee, may give way, inability to straighten
118
What is the treatment of discoid meniscus?
Knee arthroscopy
| Physio post-surgery
119
What Hb level indicates anaemia in a neonate?
Hb <140 g/L
120
What Hb level indicates anaemia in a 1 - 12 month old?
Hb <100 g/L
121
What Hb level indicates anaemia in a child aged between 1 - 12 years?
Hb <110 g/L
122
What are the mechanisms of anaemia?
Impaired red cell production
Increased red cell destruction
Increased demand
Blood loss
123
What can cause impaired red cell production?
Red cell aplasia
| Ineffective erythropoiesis
124
What can cause ineffective erythropoiesis?
Iron deficiency
Folic acid deficiency
Chronic inflammation
Rare - myelodysplasia, lead poisoning
125
What conditions can cause red cell aplasia?
```
Parvovirus B19 infection
Diamond-Blackfan anaemia
Transient erythroblastopenia of childhood
Falcon anaemia
Aplastic anaemia
Leukemia
```
126
What can cause increased red cell destruction?
```
Hereditary spherocytosis
G6PD deficiency
Thalassaemias
Sickle cell disease
Haemolytic disease of the newborn
```
127
What can cause blood loss in children?
Trauma
Feto-maternal bleeding
Chronic GI loss (Meckel's diverticulum)
von Willerbrand disease
128
What antibodies exist against blood group antigens?
Anti-D 'Rhesus"
Anti-A or anti-B
Anti-Kell
129
What is the pathophysiology of HDN?
Mother = -ve
Baby = +ve
Mother makes abs that cross the placenta and cause HDN
130
How is HDN diagnosed?
Coombs test - positive direct anti-globulin test
131
How does HDN present?
```
Pallor
HSM
Oedema
Ascites
Petechiae
Increased unconjugated bilirubin
```
132
How is HDN treated?
Prevent sensitisation with Rh immune globulins
| Intrauterine transfusion
133
What is physiological anaemia?
"Nadir"
| Reached at 2 months when cross over from HbF to HbA occurs
134
What is Falconi anaemia?
Autosomal recessive inherited aplastic anaemia
135
What is the pathophysiology of Falconi anaemia?
Mutation in FANC genes (mainly FANCA)
136
What congenital abnormalities are seen in Falconi anaemia?
```
Short stature
Abnormal radii and thumbs
Renal malformations
Microphthalmia
Pigmented skin lesions
Bone marrow failure
```
137
When are signs of bone marrow failure seen in children with Falconi anaemia?
5-6 years old
138
How is Falconi anaemia diagnosed?
Normal blood count at birth
| Chromosomal breakage test - increased chromosomal breakage of peripheral lymphocytes
139
What is the prognosis of Falconi anaemia?
High risk of death from bone marrow failure
| Transformation to acute leukaemia
140
What is the treatment of Falconi anaemia?
Bone marrow transplant
141
What percentage of iron from breast milk is absorbed?
50%
142
What percentage of iron from cow's milk is absorbed?
10%
143
What contributes to anaemia of prematurity?
Inadequate erythropoietin production
Reduced red cell lifespan
Frequent blood sampling
Iron and folic acid deficiency (2-3 months-)
144
What is sickle cell disease?
Rigid sickled cells due to AR of abnormal haemoglobin gene on chromosome 11.
145
What causes HbS to form?
Point mutation of codon 6 of B-globulin
Change in amino acid from glutamine to valine
A to G
146
What Hb is inherited in Sickle cell disease?
HbSC
| HbS from one parent, HbC from the other
147
What Hb is inherited in Sickle cell anaemia?
HbS from both parents
148
What Hb is inherited in Sickle cell trait?
HbS from one parent, normal B-globulin gene from the other (HbA)
40% HbS, asymptomatic
149
What problems are caused by HbS?
Low oxygen tension
| Cells cannot flex through capillaries leading to blockage, vessel occlusion and ischemia
150
What complications can occur with SCD?
```
Anaemia
Infection/spesis
Painful vaso-occlusive crises
Splenomegaly
Priapism
```
151
What are long-term problems associated with SCD?
```
Strokes
Short stature
Delayed puberty
Cardiomegaly and HF
Pigment gallstones
Renal dysfunction
```
152
What can precipitate vaso-occlusive crises?
Cold, infection, exercise, dehydration, hypoxia
153
How may sequestration crises present?
Sudden HSM, abdominal pain and circulation collapse
154
What should be given to prevent infection in SCD?
Prophylactic penicillin - reduced mortality due to sepsis
| Children should be fully immunised
155
How is SCD detected?
Neonatal screening by Hb electrophoresis.
156
How can vaso-occlusive crises be avoided in SCD?
Avoid cold, dehydration, excessive exercise, hypoxia
157
How would painful crises in SCD be treated?
```
Oral/IV analgesia
Fluids
Exchange transfusion
Hydroxycarbamide increases HbF
No response/stroke - BM transplant
```
158
What is thalassaemia?
Reduced globin chain synthesis
159
What is beta-thalassaemia?
Defect in beta chains
| 4 in adult haemoglobin
160
What is the inheritance pattern of beta-thalassaemia?
Autosomal recessive HBB gene on chromosome 11
161
What is the consequence of reduced B chain production in beta-thalassaemia?
Excess of alpha chains
HbA2 raised - 2 alpha, 2 delta
HbF raised - 2 alpha, 2 gamma
162
Describe beta-thalassaemia minor.
Heterozygote carrier state
Asymptomatic
Mild anaemia, low MCV, raised HbA2
163
What mutation usually occurs in beta-thalassaemia?
Point mutations
164
How is beta-thalassaemia diagnosed?
Hb electrophoresis - raised HbA2 and HbF
165
What is beta-thalassaemia intermedia?
Individuals who are symptomatic with moderate anaemia that do not require regular transfusions
166
When does beta-thalassaemia present?
First year of life
167
How does beta-thalassaemia present?
Failure to thrive
Recurrent bacterial infections
Severe anaemia at 3-6 months
HSM and bone expansion - characteristic faces
168
What is seen on blood film of beta-thalassaemia?
Large and small irregular hypochromic RBC
169
What would blood results show?
Low MCV
| Serum ferritin is normal
170
What is the treatment of beta-thalassaemia?
BT every 2-4 weeks
Iron chelating agents
Ascorbic acid
Folic acid long term
171
What are the complications of frequent blood transfusions?
Iron overload
Deposited in the liver and spleen - liver fibrosis and cirrhosis
Endocrine glands - diabetes, hypothyroidism, hypocalcaemia
172
What causes alpha-thalassaemia?
Gene deletions on chromosome 16
173
What is the clinical presentation of 4 gene deletion alpha-thalassaemia?
Only 4 gamma chains - Hb Barts - cannot carry O2
Babies still born or die shortly after
Hydrops fetalis
174
What is the clinical presentation of 3 gene deletion alpha-thalassaemia?
HbH (4 beta) disease
Moderate anaemia and splenomegaly
Not usually transfusion dependant
175
What is the clinical presentation of 2 gene deletion alpha-thalassaemia?
Carrier
Alpha-thalassaemia trait
Microcytosis
With or without anaemia
176
What is the clinical presentation of 1 gene deletion alpha-thalassaemia?
Normal blood picture
177
What is thrombocytopenia?
Low platelet count
178
What is ITP?
Destruction of platelets by IgG autoantibodies.
179
When does ITP occur?
Post viral infection in children ages between 2-10 years
180
What are petechiae?
Small red/brown/purple spots 1-2mm in size
181
What are purpura?
Purple spots from blood pooling under the skin <1cm
182
What is the acute presentation of ITP?
Petechiae on extremities
Purpura/superficial bruising
Can cause epistaxis/mucosal bleeding
183
What is the chronic presentation of ITP?
Platelet count remains low
| Associated bleeding
184
How is ITP diagnosed?
Diagnosis of exclusion
| Low platelets
185
What is the treatment of ITP?
Usually self-limiting
Rarely need prednisolone
Platelet transfusion in life-threatening haemorrhage
186
What is von Willebrand disease?
Bleeding disorder caused by abnormality in vWF, carrier protein for factor VIII
187
What is the role of vWF?
Binds to platelets and acts as bridge between damaged subendothelium.
188
Where does vWF bind?
To glycoprotein Ib on platelets
189
What types of vWD are autosomal dominant?
Type 1 - mild
| Type 2A/2B - moderate
190
What are features of vWD?
Bruising
Excessive/prolonged bleeding following surgery
Mucosal bleeding - epistaxis/menorrhagia
191
What is the treatment of vWD?
Tranexamic acid
Type 1 - desmopressin (increases vWF and factor VIII)
Type 2 - vWF/factor VIII plasma concentrates
192
Haemophilia A is a deficiency in which clotting factor?
VIII
193
Haemophilia B is a deficiency in which clotting factor?
IX
194
What is the inheritance pattern of Haemophilia?
X linked
195
What is the CP of Haemophilia in the neonatal period?
Cephalohematoma
Iatrogenic bleeding
Umbilical cord bleeding
196
What is the CP of Haemophilia in early childhood?
Easy bruising
Mouth bleeding
Spontaneous muscle/joint bleeds
197
What is the treatment of haemophilia?
Factor VIII or IX
198
What test can be done for haemophillia and what would the result be?
aPPT - prolonged
| activated partial thromboplastin time
199
What is acute lymphoblastic leukaemia?
Malignant disorder of lymphoid progenitor cells
200
What cells does ALL arise from?
Majority B cells
| Can be T cells
201
What is the pathophysiology of ALL?
Lymphoid precursors proliferate and replace normal cells of bone marrow. Blasts spill into peripheral circulation.
202
How is ALL distinguished from other malignancies of lymphoid tissue?
By immunophenotype of the cell.
| Cytochemistry and cytogenetic markers used to classify.
203
What is the peak age of ALL?
2-4 years
204
What is meant by marrow failure?
Pancytopenia
Anaemia - low Hb
Infection - low WCC
Bleeding - low platelets
205
What is the CP of ALL?
```
Those associated with pancytopenia
Fever without infection
Bone/joint pain
Early satiety
Mediastinal lymphadenopathy
```
206
How is ALL diagnosed?
FBC: anaemia, thrombocytopenia, neutropenia
Blood film: shows blast cells
Bone marrow aspirate/biopsy - confirms ALL, 50-98% nucleated cells = blasts
207
How would you confirm CNS involvement in ALL?
LP - pleocytosis (large number of lymphocytes)
208
What is the treatment for ALL?
Radiotherapy
Chemotherapy
Blood replacement therapy
209
What are the 5 stages of chemotherapy treatment for ALL?
```
Induction
Consolidation
Interim maintenance
Delayed intensification
Maintenance
```
210
What are complications of ALL?
Neutropenic sepsis
Hyperuricemia
Poor growth
Other cancers
211
What is neutropenic sepsis?
Patient with low neutrophil cannot fight infection and becomes septic
212
How is neutropenic sepsis treated?
Tazocin +/- gentamicin +/- imipenem
| Co-triomazole to prevent pneumocystis
213
Why may hyperuricemia occur? How would you prevent it?
Massive cell death at induction. Prevent with allopurinol.
214
Where does Wilms tumour originate?
Embryonal renal tissue
215
What AD mutation can cause Wilms tumour?
WT1 or WT2 gene on chromosome 11
216
What are risk factors of Wilms tumour?
Edwards syndrome
Bloom's syndrome
WAGR
217
What is WAGR?
Wilms
Aniridia - absence of iris
Gonadoblastoma
Mental retardation
218
By what age does Wilms tumour usually present?
80% by 5 years
| Rarely seen >10 years
219
What is the CP of Wilms tumour?
Haematuria
Reduction in appetite
May be asymptomatic abdominal mass
220
How is Wilms tumour diagnosed?
USS, CT, MRI
Urinalysis
Avoid renal biopsy
221
How is Wilms tumour treated?
Nephrectomy preceded by chemotherapy
| Radiotherapy if advanced
222
What is a retinoblastoma?
Cancer that develops rapidly from immature cells of the retina
223
What causes retinoblastoma to develop?
Loss of function of retinoblastoma suppressor gene on chromosome 13.
224
When is retinoblastoma usually diagnosed?
<18 months
225
What can be seen on examination?
Absent red reflex (also seen in cataracts)
Squint
Visual problems indicated
226
What is the treatment for retinoblastoma?
Enucleation
External beam radiotherapy
Chemo
Photocoagulation
227
Where do neuroblastomas arise from?
Neural crest tissue in the adrenal medulla and sympathetic nervous system
228
When are neuroblastomas most common?
< 5 years
229
What is the presentation of a neuroblastoma?
```
Pallor
Weight loss
Abdominal mass
Hepatomegaly
Bone pain
```
230
What are some less common symptoms of neuroblastoma?
```
Paraplegia
Cervical lymphadenopathy
Proptosis
Skin nodules
Periorbital bruising
```
231
What is found in the urine of a child with neuroblastoma?
Raised catecholamine metabolite levels
VMA
HVA
232
How is localised neuroblastoma managed?
Surgery
233
How is metastatic neuroblastoma managed?
Older children
| Chemo, surgery and radiotherapy
234
What are symptoms of raised ICP?
```
Headache (worse on lying down)
Vomiting
Papilloedema
Confusion
Seizures
Ataxia
Reduced consciousness
```
235
What is Cushings triad?
Physiological response to increased ICP:
Increased systolic BP
Reduced/irregular respiration
Bradycardia
236
What is Cushings triad a sign of?
Impending brain herniation
237
When should children be scanned for suspected CNS malignancy?
Headache +
| Papilloedema, neuro signs, morning vomiting, ataxia, decelerated linear growth, < 3 years, NF1
238
What is the treatment for CNA malignancy?
Surgery - resection, VP shunt
Chemo
Radiotherapy - post surgery, often used in combination with chemo
239
Why is chemo not always effective for CNS malignancy?
Many do not pass the BBB
