Paediatrics 4 Flashcards
What area are most childhood brain tumours located?
infratentorial (below the cerebrum)
What tumours most commonly spread to the brain?
Lung (most common), breast, bowel, skin, kidney
What is the most common primary brain tumour in children?
Pilocytic astrocytoma (brain tumour originating from the star shaped cells, astrocytes). They are low grade gliomas
What is the most common supratentorial tumour in children?
Craniopharyngioma
What is a craniopharyngioma?
Solid/cystic tumour of the sella region (near the pituitary gland and hypothalamus). They are slow growing midline epithelial tumours
How do infratentorial tumours typically present in children?
Raised ICP, headaches, vomiting, cerebellar ataxia
What is the best diagnostic method for brain tumors?
CT: quick and available. MRI: generally gives better tumour definition
How is hydrocephalus due to brain cancer typically treated?
Control swelling with high dose steroids (usually dexamethasone) and CSF drainage via surgery
What are the most common sites of pilocytic astrocytomas in children?
Cerebellum and optic pathway
What condition has high co-morbidity with optic pathway low grade gliomas?
Neurofibromatosis type 1
What are differential diagnosis of billious vomiting in neonates?
Duodenal atresia, malrotation with volvulus, meconium ileus
What sign is seen on AXR with duodenal atresia?
Double bubble sign
What are sanctuary sites in chemotherapy?
Areas where the chemotherapy doesn’t reach: cancer can survive. CNS and testes
What is the treatment for brain cancer?
- excision
- craniospinal radiotherapy
- chemotherapy
What is an ependymoma?
Tumour of the epndymal cells that line the ventricles
What is the most common site of ependymoma in children compared to adults?
Children: intracranial
Adults: spinal
How do ependymoma typically present?
Obstructive hydrocephalus
What population are CNS germ cell tumours typically seen in?
Teenage males
Where do germ cell tumours typically arise?
Pineal or suprasellar regions of the brain
What is the most common type of CNS germ cell tumour?
Germinoma
What are secreting tumours characterised by?
Raised markers (AFP or hCG) in serum or CSF
What are the different types of germ cell tumours?
Germinomas (pure germ cell tumours) and non germinomatous tumours (secrete chemicals into CSF + bloodstream). Types of non germinomatous tumours: terotomas, choriocarcinomas, yolk sac tumours, mixed
How are CNS germ cell tumours treated?
- teratoma: surgery
- all others: radiotherapy and chemotherapy
What hormone do teratomas secrete?
hCG; however, most do not actually secrete it
What is the most common leukaemia in children?
ALL
What is the 2nd most common leukaemia in children?
AML
What age does ALL most commonly effect?
2-3 years
What age does AML typically affect?
Under 2 years
What are risk factors for leukaemia in children?
- radiation exposure in pregnancy
- Down’s
- Kleinfelter syndrome
- Noonan syndrome
- Fanconi’s anaemia
How does leukaemia typically present?
Persistent fatigue, unexplained fever, failure to thrive, weight loss, night sweats, pallor, petechiae and abnormal bruising and unexplained bruising (due to thrombocytopenia), abdominal pain, generalised lymphadenopathy, bone/joint pain
What should be done for any child presenting with unexplained petechiae or hepatomegly?
Immediate specialist assessment
What is the management for a child presenting with queried leukaemia?
Full blood count in 48 hours
What might be seen on a blood film in leukaemia?
Blast cells
What is the management of leukaemia?
Primarily chemotherapy. Can also do radiotherapy, bone marrow transplant, surgery
What are poor prognosis factors for CLL?
- <2 years or >10 years
- WBC >20*10^9/l at dx
- T or B cell surface markers
- non caucasian
- male sex
What condition might be associated with gingivial hyperplasia?
AML
What is Wilms’ tumour?
A tumour affecting the kidney in children, generally under 5
What is the presentation of Wilms’ tumour?
Consider in any child under 5 presenting with a mass in the abdomen. Also:abdominal pain, haematuria, lethargy, fever, HTN, weight loss
What is the first line investigation for Wilms tumour?
Ultrasound. Then CT/MRI to stage, and biopsy for histology
What is the management of Wilms’ tumour?
Surgical excision of the tumour along with the affected kidney (nephrectomy). May also give adjuvant chemo or radiotherapy
What is prognosis of Wilms tumour?
Good
If a child presents with an unexplained abdominal mass, what is the next steps?
Urgent paediatric review within 48 hours
What is a neuroblastoma?
A tumour arising from the neural crest tissue of the adrenal medulla and sympathetic nervous system
What are the features of neuroblastoma?
Abdominal mass, pallor, weight loss, limp, bone pain, hepatomegaly, paraplegia, proptosis
What is a retinoblastoma?
Ocular malignancy
What is the average age of diagnosis of retinoblastoma?
18 months
What is the cause of retinoblastoma?
Can be sporadic, but there are familial forms that are autodomal dominant
What is the presentation of retinoblastoma?
absence of red reflex of the eye (leukocoria), strabismus, visual problems
What is the management?
Treatment may involve radiotherapy or enucleation (removal of the eye), with chemotherapy
What cancer is associated with auer rods?
AML
What is the most common malignant bone tumour?
Osteosarcoma
Where do osteosarcomas most commonly occur?
In th elong bones prior to epiphyseal closure
What is seen on xray for osteosarcomas?
Codman triangle and a sunburst pattern
What tumour may be associated with retinoblastoma?
Osteosarcoma: both have mutation of Rb gene
Where does Ewing’s sarcoma typically occur?
In the pelvic and long bones
What is seen on a xray in Ewing’s sarcoma?
Onion skin appearance
What is the presentation of osteosarcoma?
Localised pain and swelling, pathological fracture
What lobe of the liver does a hepatoblastoma typically begin in?
Right
What is tumour lysis syndrome?
When chemicals are released when cells are destroyed by chemo. Results in: high uric acid, high potassium, high phosphate, low calcium
What can reduce tumour lysis syndrome?
Allopurinol can reduce uric acid. Can also reduce AKI
What age is Hodgkin’s lymphoma most common?
Bimodal peaks: 20-25 and 80
What are risk factors for hodgkins lymphoma?
HIV, EBV, autoimmune conditions (such as rheumatoid arthritis and sarcoidosis), FHx
What are three types of non-hodgkins lymphoma?
Diffuse large B cell lymphoma, Burkitt Lymphoma, MALT lymphoma
What types of Non-Hdgkins lymphoma are particularly associated with HIV?
Burkitt
What are risk factors for non-hodgkins lymphoma?
HIV, EBV, H.pylori infection, Hep B or C, pesticides exposure, FHx
What are B symptoms of lymphoma?
Fever, weight loss, night sweats
What unique symptom will patient’s with Hodgkin’s lymphoma experience?
Lymph node pain after drinking alcohol
What is a typical finding for Hodgkin’s lymphoma on biopsy?
Reed-Sternberg cells: owl cells
What is the classification used for lymphoma?
Lugano classification: replacing the old Ann Arbor system
What are b symptoms associated with in Hodgkin’s lymphoma?
Poor prognosis
What is each of the stages of lymphoma?
1: single node/group of nodes
2: more than one group of nodes but on same side of the diaphragm
3: multiple nodes but both sides of the diaphragm
4: widespread involvement and symptoms outside fo the lymph nodes
What does rituximab target?
B cells
What are the two chemotherapy regimes that are used in Hodgkins lymphoma management?
ABVD (standard). Also BEACOPP.
Which type of lymphoma is most common?
Non-hodgkins
Which type of lymphoma more commonly has extra nodal disease?
Non hodgkins
What is seen on biopsy for Burkitt’s lymphoma?
‘Starry sky’ appearance
What are the steps of newborn resuscitation?
- Dry baby and maintain temperature
- Assess tone, RR, HR
- If gasping or not breathing give 5 inflation breaths
- Reassess chest movements
- If the heart rate is not improving and <60 BPM start compressions and ventilation breaths at a rate of 3:1
What is assessed in an apgar score?
Appearance (skin colour), pulse, grammace (response to stimulation), activity (muscle tone), respiration
What is the compression to ventilation ratio in PBLS?
If one person, 30:2, and if two people, 15:2
What is the rate of chest compressions in PBLS?
100-120
What are risk factors for neonatal sepsis?
Vaginal GBS colonisation, GBS sepsis in a previous baby, maternal sepsis/chorioamnionitis/fever, prematurity (<37 weeks), early rupture of membranes, PROM, low birth weight
What are red flags for potential neonatal sepsis?
Confirmed or suspected sepsis in the mother, signs of shock, seizures, term baby needing mechanical ventilation, respiratory distress starting more than 4 hours after birth, presumed sepsis in another baby in multiple pregnancy
What is the most common presentation of neonatal sepsis?
Respiratory distress, such as grunting, nasal flaring, use of accessory respiratory muscles, tachypnoea
How is diagnosis usually established in neo natal sepsis?
Blood culture
What baseline investigations should be done for neonatal sepsis?
FBC and CRP
What abx are used in treatment of sepsis/suspected sepsis?
BenPen and gentamycin
What can acidosis on the umbilical artery blood gas indicate in a newborn?
Hypoxic Ischaemic Encephalopathy
What are the risk factors for necrotising enterocolitis?
Low birth weight/premature, formula feeds, respiratory distress and assisted ventilation, sepsis, patent ductus arteriosus and other congenital heart conditions
What is the presentation of necrotising enterocolitis?
Intolerance to feeds, vomiting (particularly with green bile), generally unwell, distended abdomen, absent bowel sounds, blood in stools
What are signs seen in xray for necrotising enterocolitis?
Dilated loops of bowel, bowel wall oedema, pneumatosis intestinalis (gas in bowel wall), free gas in the peritoneal cavity
What is the management of necrotising enterocolitis?
NBM with IV fluids, total parenteral nutrition and abx. Surgical emergency
What population is bronchopulmonary dysplasia seen in?
Premature: often before 28 weeks
What is the diagnosis of bronchopulmonary dysplasia based upon?
- chest xray changes
- infants requiring oxygen therapy after 36 weeks
What are the features of bronchopulmonary dysplasia?
Low O2 saturations, increased work of breathing, poor feeding and weight gain, crackles and wheezes on chest ausculatation, increased susceptibility to infection
What is the preventative method for bronchopulmonary dysplasia?
Give corticosteroids if in premature labour before 36bweeks
What can be done when a newborn is born to prevent development of bronchopulmonary dysplasia?
- CPAP rather than intubation and ventilation
- caffeine to stimulate respiratory effort
- not over oxygenating