Paeds 1 Flashcards
What is the management of Scarlet fever?
Antibiotics
Rest, fluids, good hygiene to prevent spread.
Notify the local PHE centre.
What causes the rash in scarlet fever?
the Exotoxin (produces by step pyogenes)
What is the causative organism for Scarlett fever?
Streptococcus Pyogenes
What deficiency can cow’s milk cause and how?
iron deficiency
- low content of iron in cow’s milk
- can cause intestinal blood loss (in 40% infants)
What causes acute epiglottis?
Haemophilus B influenza (Hib vaccine protects against this)
Who is immune to parvovirus infection and why?
Individuals lacking erythrocyte P antigen
this is because parvovirus replicates in red cell precursors expressing P antigen
Who is at particular risk of Parvovirus infection?
a) people with haemoglobinopathies, e.g. SCD - are at greater risk of aplastic crisis with parvovirus infection
b) immunocompromised individuals - can develop chronic infection
c) pregnant women - rarely, parvovirus infection in the first 20 weeks of pregnancy can cause miscarriage or congenital abnormalities if the woman is not immune.
What rash is characteristic of parvovirus infection?
bright red cheeks;
can cause a lace-like rash in the trunk and arms, more in women and adults; my itch, partculrarly after a hot bath;
What is hydrops fetalis?
a life-threatening condition in which abnormal amounts of fluid accumulate in two or more body areas of an unborn baby. Although the fluid buildup may appear anywhere in the baby’s body, it most often occurs in the abdomen, around the heart or lungs, or under the skin.
standard approach to parvovirus diagosis
serology
How is malaria diagnosed?
blood film
What does the APTT blood test examine?
intrinsic pathway of blood clotting (factors: VIII (and vWF), IX, and XI specifically.
what does aPTT stand for?
activated partial thromboplastin time
What alterations in vaccination should be made in patients with haemophilia?
stick to the vaccination schedule but give the vaccinations s.c. rather than i.m.
What common medication should be avoided in haemophilia and why?
NSAIDs because they can affect platelet function
What is another name for henoch-schonlein purpura?
IgA vasculitis
What is the most common vasculitic disease in childhood?
Henoch Schönlein Purpura
Tetrad of HSP
- rash
- abdominal pain
- arthritis/arthralgia
- glomerulonephritis
What are common complications of HSP?
Intussusception
Acute renal impairment
Arthritis/arthralgia, typically involving ankles and knees
Pancreatitis
Testicular/scrotal involvement is reported in a minority of cases in boys.
Why do you have to hyperhydrate patents with high WCC leukemias?
TLS is a risk; TLS can progress to renal failure;
Therefore hyper hydration and allopurinol should be commenced immediately.
Levels of what can be increased in cancers with high cell numbers or a high mass and why?
potassium, phosphate and nuclear debris (causing high uric acid and LDH
Any cancer with a high number of cells or a high mass, will undergo lysis resulting in release of these.
First line management of acute asthma?
- high flow oxygen (10-15L/min via non-rebreather mask)
- inhaled salbutamol
5mg (2.5 mg in children under 5) in 4ml 0.9% NaCl with 6-8 L/min O2.
What is HSP often preceded by?
viral URTI
What % of meconium ileus are associated with CF?
90%
Why is uric acid high in TLS?
Due to breakdown of genetic material
Management of TLS
hyperhydration
crystalloid (0.9% NaCl with 5% dextrose) -> no K+ because levels are high in TLS
allopurinol
complication of TLS
renal failure due to phosphate and uric acid precipitating in the tubules.
Why do you hyperhydrate in TLS?
to dilute the phosphate and potassium in the blood
also to make sure the kidneys are perfused?
What fluid to you give in TLS to hyperhydrate in paediatrics?
Crystalloid: 0.9% NaCl with 5% dextrose to ensure constant
avoid potassium in the bag because in TLS the levels of potassium are high
give fluids according to weight
What does allopurinol do and how?
decreases level of uric acid by inhibiting XO oxidase which is involved in the breakdown of purines into uric acid
What does allopurinol do and how?
decreases level of uric acid by inhibiting XO oxidase which is involved in the breakdown of purines into uric acid (pg 42 top drugs)
Hyperleucocytosis
thick blood due to high number of white cells
here be careful when transfusing e.g. platelets because you do not want to make the blood more viscous; only give small amounts.
Paedatric oncological tertiary centres in London
north: GOSH for young children, UCLH for approx above 13
south: royal Marsden
Febrile neutropenia
anyone with Neutrophils <0.5 and >38
RISK: (gram -ve) neutropenic sepsis
Why do children with Leukemia get Cotrimoxazole every week?
to prevent opportunistic PCP
What do you give to prevent line infection?
teicoplanin (against gam +ve)
What systems does HSP usually affect?
skin, GI tract, renal, joints
typical joint problems in HSP
bilateral large join swelling (ankle, knee, wrist) -> treat with NSAID eg ibuprofen
urine dipstick in HSP dipstick renal failure? what other tests to do?
(microscopic) haematuria and proteinuria
Cr, UREA, BP
Where in London are children with haemophilia treated?
GOSH
when grow up: royal free
What are common indications for allopurinol?
- prevent recurrent attacks of gout
- prevention of uric acid and calcium oxalate real stones
- to prevent hyperuricaemia and TLS ass. with chemotherapy
MOA allopurnol
xanthine oxidase inhibitor
What are the most common childhood cancers in the UK?
- Leukemia (32%)
- Brain and spinal tumours (24%)
- Lymphomas (10%)
- Neuroblastoma (7%)
- Soft tissue sarcoma (7%)
- Wilms tumour (6%)
- Bone tumours (4%)
- Retinoblastoma (3%)
- Other (7%)
Example of an inherited childhood cancer (incl genetic mutation)
bilateral retinoblastoma (mutation within RB ? chromosome 13)
What cancers is Down syndrome associated with?
leukemia
neurofibromatosis
glioma
What are the most common childhood cancers in Africa?
Burkitts lymphoma and kaposis sarcoma
what are examples of infection related cancers and what pathogens are they associated with?
Burkitt’s lymphoma, hodgkin lymphoma, nasopharyngeal carcinoma - EBV
liver carcinoma - hep B
Kaposis sarcoma - HIV and human herpesvirus 8
what are examples of infection related cancers and what pathogens are they associated with?
Burkitt’s lymphoma, hodgkin lymphoma, nasopharyngeal carcinoma - EBV
liver carcinoma - hep B
Kaposis sarcoma - HIV and human herpesvirus 8
levels of what in urine can be useful in confirming the diagnosis of neuroblastoma?
increased urinary catecholamine excretion (VMA - vanillylmandelic acid; HVA - homovanillic acid)
When is a-fetoprotein increased?
in liver tumours and in germ cell tumours
What translocation in seen in Ewing sarcoma?
chromosomes 11 and 22
Cancer survival of teenagers+young adults vs children
teenagers and young adults have poorer outcomes
what do ALL and CLL stand for?
acute lymphoblastic leukemia and chronic lymphocytic leukemia
What is the problem with radiotherapy in children?
- the risk of damage to growth and function of normal tissue is greater in a child than in an adult
- practical difficulties due to the need for adequate protection of normal tissues, careful positioning and immobilisation of the patient during treatment
- cranial radiotherapy in children <3yo -> significant risk of severe damage to neurocognitive development
Summarise the important short term side effects of chemotherapy in children
BM suppression -> anaemia, thrombocytopenia and bleeding, neutropenia (-> infection)
Immunosuppression -> Infection
Gut mucosal damage -> infection (particularly gram-ve) and undernutrition, diarrhoea
N&V -> undernutrition
anorexia -> undernutrition
alopecia
What to do if a child presents with fever and neutropenia?
admit promptly to hospital for cultures and treatment with broad-spectrum antibiotics
What are some opportunistic infections commonly seen in children that are immunocompromised due to cancer (therapy)?
- Pneumocystis jiroveci (carinii) pneumonia (especially in children with leukemias
- disseminated fungal infection e.g. aspergillosis and candidiasis
- coagulase-negative staphylococcal infections of central venous catheters
What vaccines are contraindicated during and following cancer treatment and how long?
live vaccines are CI’d during chemotherapy and for 6-12 months subsequently due to depressed immunity.
After this period re-immunisation against common childhood infections is recommended.
What viral infections are dangerous for children undergoing chemotherapy?
- Measles
- Varicella Zoster
-> may have atypical presentation and may be life-threatening
If at risk of contact with measles or chickenpox, some protection can be afforded by prompt administration of immunoglobulin or zoster immune globulin.
Aciclovir is used to treat established varicella infection but no treatment is available for measles.
Most other viral infections are no worse than in other children.
What are the options for venous access in chemotherapy?
-tunnelled venous catheter (Hickman lines)
- implantable ports (=left entirely under the skin)\central venous catheters can remain in situ for many months if not years but carry the risk of infection and can get blocked or split.
What are the options for venous access in chemotherapy?
-tunnelled venous catheter (Hickman lines)
- implantable ports (=left entirely under the skin)
central venous catheters can remain in situ for many months if not years but carry the risk of infection and can get blocked or split.
What is the peak age of ALL presentation?
2-5
What is the commonest leukemia in children?
ALL -> makes up 80% of all childhood leukemias
followed by AML and ANLL (acute nonlymphocytic leukemia)
Why are steroids given when treating leukemia?
x
Treatment of leukemia
- make sure anaemia, thrombocytopenia and TLS are sufficiently under control before starting chemotherapy
- remission induction with combination chemotherapy including steroids
- Intensification: a block of intensive chemotherapy is given to consolidate remission (better cure rates but higher toxicity)
- CNS - cytotoxic drugs poorly penetrate the CNS; leukaemia cells at this site may survive effective treatment, therefore intrathecal chemotherapy is given to prevent CNS relapse; patients with CNS disease evidence at presentation are given additional doses of intrathecal chemotherapy during induction.
- continuing therapy at modest intensity for a relatively long time period up to 3 years from dx; cotrimoxazole prophylaxis is given to prevent PCP
What is given to prevent PCP in leukemia?
prophylactic cotrimoxazole
What is co-trimoxazole and what is it used for?
Trimiethoprim with sulfonamide e.g. sulfamethoazole
prevention of PCP in immunosuppression, e.g. HIV or leukemia or chemotherapy
(trimethoprim ought to have broad activity against gram +ve and -ve bacteria; combination with sulphonamide extends the spectrum to include activity against the fungus causing PCP
What are the most common solid tumours in children?
brain tumours
What is different about brain tumours in children compared to adulrs
in children they are almost always primary rather than metastatic and 60% are infratentorial (located below the tentorium cerebelli)
What is the leading cause of childhood cancer deaths?
brain tumours
Liste the types of brain tumours in children
- astrocytoma (40%) - varies from benign to highly malignant (glioblastoma multiforme)
- medulloblastoma (20%) - arises from the midline of the posterior fossa
- ependymoma (8%) - mostly in posterior fossa where it behaves like medulloblastoma
- brainstem glioma (6%) - malignant tumours associated with a very poor prognosis
- craniopharyngioma (4%) - developmental tumour arising from the squamous remnant of Rathke pouch; not truly malignant but locally invasive.
- atypical teratoid/rhabdoid tumour - a rare type of aggressive tumour that most commonly occurs in young children
Which lymphomas are more common in childhood and which in adolescence?
NHL is more common in childhood;
Hodgkin lymphoma is more common in adolescence.
What causes neonatal respiratory distress syndrome?
lack of surfactant
where do neuroblastoma and related tumours arise from?
neural crest tissue in the adrenal medulla and sympathetic NS
how does neuroblastoma present?
most children have an abdominal mass, but the primary tumour can lie anywhere along the sympathetic chain from the neck to the pelvis;
classically the abdominal primary is of adrenal origin but at presentation the tumour mass is often large and complex, crossing the midline, enveloping major blood vessels and lymph nodes.
At what age is neuroblastoma most common?
Under the age of 5
How is neuroblastoma diagnosed?
- characteristic clinical and radiological features
- increased levels of urinary catecholamines
- confirmatory biopsy
- evidence of metastatic disease with BM sampling and MIBG scan
Management of neuroblastoma
- localised primaries without metastatic disease can often be cured with surgery alone
- in some infants neuroblastoma (incl. metastatic) may resolve spontaneously
- metastatic disease in older children is treated with chemotherapy (incl. high dose therapy with autologous stem cell rescue, surgery and radiotherapy)
high risk of relapse
Where does Wilms tumour originate from?
embryonal renal tissue
what is the most common renal tumour of childhood?
Wilms tumour
In what age groups is Wilms tumour seen?
over 80% present before 5yo and it is rarely seen after 10yo
Clinical presentation and features of Wilms tumour
most children present with large abdominal mass (and are otherwise well)
common: abdo mass, haematuria
uncommon: abdo pain, anorexia, anaemia (haemorrhage into mass), hypertension
Where does Wilms tumour normally metastasise to?
lungs
Investigations for Wilms tumour
US +/- CT/MRI usually characteristic with intrinsic renal mass distorting normal structure.
staging to assess distant mets, initial tumour respectability and function of contralateral kidney
Management of Wilms tumour
- initial chemotherapy
- delayed nephrectomy -> histological staging, surgical and path findings helps plan further management
- radiotherapy in more advanced disease
in bilateral disease (5%) particularly careful management to preserve as much renal function as possible
What % children with Wilms tumour present with bilateral disease?
5%
prognosis of Wilms tumour
good
> 80% pts cured
with metastatic disease (15%) the cure rate is >60%
relapse carries a poor prognosis
What is the most common soft tissue sarcoma in childhood?
Rhabdomyosarcoma
What tissue does rhabdomyosarcoma originate from?
primitive mesenchymal tissue
Are bone tumours in children more common in girls or boys?
boys
are malignant bone tumours more common before or after puberty?
after
what is more common - osteosarcoma or Ewing sarcoma?
osteosarcoma is more common but Ewing sarcoma is more common in younger children.
Ewing sarcoma is the second most common
what is more common - osteosarcoma or Ewing sarcoma?
osteosarcoma is more common but Ewing sarcoma is more common in younger children.
Clinical presentation of bone tumours
persistent, localised bone pain -> indication for early x-ray.
at dx most patients are otherwise well.
investigations in bone tumours
plain x-ray -> MRI -> bone scan
Chest CT to asses for lung mets and BM sampling to exclude marrow involvement
Management of bone tumours in children
- combination chemotherapy
- surgery: avoid amputation wherever possible with en bloc resection of tumours with end-prosthetic resection
radiotherapy in Ewing sarcoma in local disease, especially if surgical resection is impossible or incomplete e.g in pelvis or axial skeleton.
Define retinoblastoma
rare, malignant tumour of retinal cells
accounts for 5% of visual impairment in children; may affect one or both eyes.
What % of bilateral and unilateral retinoblastoma tumours are hereditary?
100% of bilateral
20% of unilateral
On which chromosome is the retinoblastoma susceptibility gene? What is the inheritance pattern?
13
dominant (but with incomplete penetrance)
When do pts with retinoblastoma usually present?
usually in the first 3 years of life
clinical features of retinoblastoma
white pupillary reflex
investigations for retinoblastoma
MRI and examination under anaesthetic; tumours are frequently multifocal.
no biopsy is done, mx based on ophthalmological findings
management of retinoblastoma
aim: cure yet preserve vision
- enucleation of the eye may be necessary for advanced disease
- chemotherapy (particularly in bilateral disease) to shrink tumour followed by local laster treatment to the retina
- radiotherapy may be used in more advanced disease but is usually reserved for recurrence.
most patients are cured but many visually impaired.
How do children usually present with Kaposis sarcoma? How is it diagnosed and treated?
generalised lymphadenopathy
dx requires biopsy confirmation
Mx with chemotherapy and ART
triggered by HHV-8
What cells is kaposi’s sarcoma derived from?
blood and lymph vessel cells
What are the common childhood cancers according to age groups?
Pre-school (<5)
- ALL (peak incidence)
- NHL
- neuroblastoma
- wilms tumour
- retinoblastoma
School-aged
- ALL
- brain tumours
Adolescnece
- ALL
- hodgkin lymphoma
- malignant bone tumours
- soft tissue sarcomas
What part of the brain is affected in cerebral palsy?
cerebral cortex (motor cortex in particular) and other components of the brain involved in strength, movement and muscle tone.
What are the patterns of cerebral palsy?
- increased tone(hypertonia, usually spasticity)
- decreased tone also possible (hypotonia, atonia)
- dysregulated movement (ataxia)
- altered movements (dyskinesia)
- combination/mixed pattern
What types of cells are UMNs usually?
pyramidal cells
What happens with muscle tone in UMN and LMN lesions?
UMN: hypertonia
LMN: hypotonia
What is the relationship of UMN and cerebral palsy?
alteration of UMN physiology can result in impaired volitional motor control and loss of UMN’s inhibitory effects on LMN.
-> loss of ‘braking’ action of UMN in LMN
-> impairment of normal movement initiation
-> impairment of smooth recovery of motor action
Predominance of uninhibited LMN function in cerebral palsy resulting in spasticity/increased tone, weakness, and hyperreflexia.
What proportion of CP are due to hypoxic-ischaemic encephalopathy (HIE)?
10-20%
What proportion of CP are due to hypoxic-ischaemic encephalopathy (HIE)?
10-20%
What are the indications to prescribing steroids?
- treatment of allergic or inflammatory disorders e.g. anaphylaxis or asthma
- treatment of autoimmune disease e.g. IBD or inflammatory arthritis
- cancer treatment (as part of chemotherapy or to reduce tumour-related swelling)
- hormone replacement in adrenal insufficiency or hypopituitarism
MOA of steroids
- bind to ic GC recentres, alter gene expression
- increase anti-inflammatory actions
- reduce pro-inflammatory actions such as cytokines and TNF-alpha
mainly GC effects.
MC effects are increased retention of Na+ and water and excretion of K+ in the renal tubules
What are ossification centres?
areas where bone tissue develops
In what order do secondary ossification centres in the elbow develop and at what ages?
CRITOL / CRITOE
capitulum
radial head
internal (medial) epicondyle
trochlea
olecranon
lateral/external epicondyle
1,3,5,7,9,11
or
1, 5,7,10,10,11 (more accurate)
What are the different parts of long bones and where are growth plates found?
diaphysis - shaft
metaphysis - between d and e
epiphysis - at the end of the bone
growth plate is between m and e
How can you classify epiphyseal fractures?
Salter Harris
I - epiphyseal slip only
II - fracture through epiphyseal plate with triangle of shaft attached
III - fracture through epiphysis extending into epiphyseal plate
IV - fracture of epiphysis and shaft, crossing the epiphyseal plate
V - crush injury
(see image on google)
Considerations for terminating continuous steroid therapy?
sudden withdrawal can lead to Addisonian crisis with cardiovascular collapse.
This is because exogenous steroids suppress pituitary ACTH, switching off the stimulus for corticosteroid production and lead to adrenal atrophy.
Withdraw slowly for gradual return off adrenal function.
Symptoms of chronic glucocorticoid deficiency on exogenous steroid withdrrawl
fatigue
arthralgia
weight loss
SE of glucocorticoids
- Immunosuppression
- diabetes mellitus
- osteoporosis
- proximal muscle weakness
- skin thinning
- easy bruising
- gastritis
- mood and behavioural changes (insomnia, confusion, psychosis, suicidal ideas)
- hypertension
- hypokalaemia
- oedema
In which groups of people should you be careful when giving steroids?
- in people with infection
- in children (in whom it can suppress growth)
Name important interactions of steriods
- NSAIDs: in combination, increase the risk of peptic ulceration and GI bleeding
- b2-agonists, theophylline, loop or thiazide diuretics: enhance hypokalaemia
- efficacy reduced by cytochrome p450 inducers (phenytoin, carbamazepine, rifampicin)
- vaccines: corticosteroids reduce immune response to them
how should OD steroids be taken?
in the morning to mimic the circadian rhythm and reduce insomnia
What advice should you give patients on steroid replacement therapy?
Double the dose when feeling ill, reduce back to maintenance dose on recovery
Which steroids are most potent?
Dexamethasone (no MC activity)
Fludrocortisone
methylprednisolone
Prednisone/Prednisolone
Hydrocortisone
what medications may need to be co-prescribed in steroid therapy?
PPIs and bisphosphonates to limit adverse effects
What is in the 6in1 vaccine?
diphtheria, tetanus, pertussis (whooping cough), Hib, Hep B, polio
What is in the 4in1 vaccine and when is it given?
pre-school booster
diphtheria
tetanus
polio
whooping cough/pertussis
What is in the 3in1 vaccine and when is it given/
diphtheria
tetanus
polio
teenage booster (14 yo)
What vaccines are given at 14 years?
3in1
MenACWY
What vaccinations are given at 2 years?
Hib/MenC
MMR
PCV (pneumococcal)
MenB
What vaccines are given at 3 years and 4 months?
4in1 pre-school booster (Diphtheria, Tetanus, Polio, pertussis)
MMR (2nd dose)
What vaccines are given at 1 year?
Hib/MenC (1st dose)
MMR (1st dose)
Pneumococcal (PCV) vaccine (2nd dose)
MenB (3rd dose)
What vaccines are given before 1 year of age?
8 weeks
6-in-1 vaccine
Rotavirus vaccine
MenB
12 weeks
6-in-1 vaccine (2nd dose)
Pneumococcal (PCV) vaccine
Rotavirus vaccine (2nd dose)
16 weeks
6-in-1 vaccine (3rd dose)
MenB (2nd dose)
What is the Abx of choice in acute otitis media?
amoxicillin
What are the indications for prescribing antibiotics (amoxicillin) in otitis media in children?
Eardrum is perforated
< 2 years old and bilateral infection
Present for ≥4 days
< 3 months old
CREAM acronym for kawasaki disease
C - conjunctivitis
R - rash
E - edema/eryhthema of hands
A - adenopathy (most commonly cervical, usually unilateral)
M - mucosal involvement (strawberry tongue, oral fissures etc)
Fever!!
What is tetralogy of Fallot?
Tetralogy of Fallot is a relatively rare form of congenital cardiac disease. It is cyanotic.
- Ventricular septal defect (VSD)
- Overriding aorta - i.e., an aorta that overrides the right and left ventricles as a result of the VSD
- Right ventricular outflow tract obstruction (RVOTO) - this is the main determinant of the severity of cyanosis
- Right ventricular hypertrophy
How do you calculate maintenance fluids required in children?
1st 10kg of bodyweight at 100ml/kg/day
2nd 10kg of bodyweight at 50ml/kg/day
Remaining bodyweight at 20ml/kg/day
The fluid type routinely used is 0.9% sodium chloride + 5% dextrose. Potassim is added as required depending on their U&Es.
What gastro condition is prematurity a RF for and what is a typical radiological finding suggestive of this condition?
Necrotising Enterocolitis
pneumatosis intestinalis on abdo X-ray
What is montelukast?
Monteleukast is an LTRA (leukotriene receptor antagonists) which works by downregulating inflammatory leukotrienes, and can be used an add-on therapy.
What is the drug known as Ritalin and what is it used for?
Methylphenidate
used for ADHD
What is the formula for calculating height velocity?
(height now - height last visit) / (age now - age last visit)
expressed in cm/year
measured at 6 month intervals
What controls growth?
GH - IGF-1 axis
What type of hormone is GH? (biochem)
single polypeptide
Which cells release growth hormone?
somatotrophs in the anterior pituitary gland
In what manner is GH released and what influences its release?
pulsatile
sleep, exercise, stress, nutrition, age (children produce more than adults)
