W08 - PAEDS: Oncology; Rheumatology Flashcards
Epidemiology & Classification
*based on tumour morphology and primary site
commonest:
- leukemias
- CNS tumours
- lymphomas
- soft tiss. tumours
- neuroblastomas
- 0-4yo highest distribution and prevalence
- 5 - 14 similar distribution
- genetic predisposition: trisomy 21; fanconi; BWS, neurofiromatosis
- radiation & infection
- iatrogenic: chemoT, RT
Red Flag Symptoms / who to be worried about
immediate referral:
* unexplained petechiae
* hepatosplenomegaly
~ leukemia; ~ NH lymphoma
urgent referral:
* rpt attendance, same problem, nil dx.
* new neuro symptoms
* abdo masss
refer:
* rest pain, back pain, unexplained lump
* lymphadenopathy
Important Emergencies
Sepsis / febrile neutropenia
Raised ICP
Spinal cord compression
Mediastinal mass
Tumour lysis syndrome
Sepsis / febrile neutropenia
pseduomonas aeruginosa
e coli
klebsiella
s pneumoniae
- fever, rigors, drowsiness, SHOCK
> IV accss
ABC
broad spectrum
urine / blood / swab culture investigations
Raised ICP
Early
early morning headache/vomiting
tense fontanelle
increasing HC
Late
constant headache
papilloedema
diplopia (VI palsy)
Loss of upgaze
neck stiffness
status epilepticus,
reduced GCS
Cushings triad (low HR, high BP)
- CT = screening
- MRI = accurate dx.
> DEXAMETHASONE IV dt tum.
Sx. urgent CSF diversion
- ventriculostomy
- EVD
- VP Shunt
Spinal cord compression
Compl of most paediatric malignancies
* Invasion from paravertebral disease via intervertebral foramina (40 % extradural)
* vertebral body compression
- weakn., pain, sensory, sphincter disturbance symptoms
> urgent MRI
DEXAMETHASOME = reduce oedema
ChemoT
SVC Syndrome
Obstruction of sup. vena cava dt mediastinal mass dt
- LYMPHOMA
- neuroblastoma, germ cell tumour, thrombosis
- facial, neck and upper thoracic plethora oedema, cyanosis, distended veins, ill, anxious, reduced GCS
> CXR / CT chest
ECHO
keep upright!; urgent biopsy
>Definitive tx: ChemoT, RT, Thrombotic Rx.
Tumour Lysis Syndrome
- metab. derangement, release of intracellular contents of lysed tumour cells
- 2º to TREATMENT - rarely spontaneous
↑ potassium
↑ urate, relatively insoluble
↑ phosphate
↓ calcium
Acute renal failure
Urate load
CaPO4 deposition in renal tubules
*ecg, IV hydration = avoid tumour lysis
> diuresis
never give K+
↓uric acid: allopurinol; urate oxidase-uricozyme
> hyperkalemia: salbutamol, insulin, calcium gluconate, calcium resonium
> dialysis
Information oncologists require and investigate for
- harm and risks:
- scans => MRI mainstay in paeds oncology
- biopsy
- cytogenetics
- tumour markers
- staging: CT, biopsy, PET
Risks of treatment
ChemoT
- hair loss, NV, mucositis, Diarrhoea, BM suppr., bleeding, infection
- organ impairment, reduced fertility
RT
- lethargy, skin irritation, swelling
- fibrosis /scarring
- 2º scarring
Describe the epidemiology of Juvenile Idiopathic Arthritis (JIA)
*commonest
- multifactorial
- immune response, pro-inflamm markers, presence of Ab.
- ARTHRITIS FOR AT LEAST 6W*
- morning stiffness
- irritability or refusal to walk in toddlers
- Phsycial activity = impact on school
+ periarticular edema, TENOSYNOVITIS
+ inflamm upset picture
+ poor appetite wt loss
+ delayed pub. - limited motion
Be aware of the medical and surgical differential diagnosis of a limp/joint pain in children
a
To recognise transient synovitis and its self-limiting nature
Transient synovitis is an inflammation in the hip joint that causes pain, limp and sometimes refusal to bear weight. This occurs in pre-pubescent children and is the most common cause of hip pain. It occurs when a viral infection, such as an upper respiratory infection, moves to and settles in the hip joint.
Recognise the clinical manifestations and types of JIA
1) Psoriatic Arth: FHx 1º relative, nail pitting, dactylitis, achilles
2) Systemic Arth: min. 2w. and 1+ joint, unwell, intermittent fever
+ erythematous rash; lymphadenopathy; hepato/splenomegaly; serositis
3) Oligorth.: 4 or fewer joints during first 6mos of disease
- girls 1-5yo pres. = ANA+; uveitis; knees ankles hands; NO HIP
- boys 8yo+ pres = ANA-; hip involvement
4) Polyarth.: 5+ joints, fewer systemic manifestations
F>M
seropos. = 8yo+
seroneg. = under 5yo
* temporomandibular joint injury = functional bite affected
* large fast growing joints mostly affected
5) ENTHESITIS:
hla, boy 8yo+, uveitis, spinal pain, sacroiliac joint, FHx
Discuss the principles of investigation and assessment of JIA and joint disease
Plain XR
US
MRI
Understand the principles of management of JIA and other inflammatory joint pathologies including long-term rehabilitation.
> NSAIDs
> DMARDs,
-mtx. commonly used. poor response in oligo., s/c form
- earlier = better outcome
- monitoring bloods
> biologics: resistant to DMARDs, anti-TNF
> oral and intra-articular steroids
- greater success in oligoarth.
> psychosocial
> school adjustments and support
> nutrition: anemia and generlaised osteoporosis
> physio
> OT
Compl. of JIA
Uveitis, risk to progressing to chronic uveitis.
- thus screening w/ every dx.
- more common in ANA+ and <5yo
*red eyes, headache, reduced vision
* slit lamp examination
!Cataracts, glaucoma and blindness
> topical steroids => systemic steroids
DMARDs
Biologics
+ poor growth
+ localised growth disturbances
+ micrognathia
+ contractures
