Revision - HUS, Wilm's Tumour, FebCon & Epilepsy Flashcards
Give 3 causes of HUS
1) E. coli 0157
2) Pneumococcal infection
3) HIV
What triad of features is seen in HUS?
1) Microangiopathic haemolytic anaemia
2) AKI
3) Thrombocytopenia
What increases the risk of HUS in gastroenteritis?
Use of antibiotics & antimotility agents (e.g. loperamide)
What causes thrombocytopenia in HUS?
The formation of blood clots consumes platelets, leading to thrombocytopenia.
What is typically the first symptom of HUS?
Diarrhoea that turns bloody within 3 days
Features of HUS?
- bloody diarrhoea
- abdo pain
- jaundice
- pallor
- fever
- lethargy
- oliguria
- haematuria
- HTN
- bruising
- confusion
Management of HUS?
MEDICAL EMERGENCY!
Admit and treat:
1) Hypovolaemia e.g. IV fluids
2) HTN
3) Severe anaemia e.g. blood transfusion
4) Severe renal failure e.g. haemodialysis
Which type of PKD usually affects adults and which affects children?
Autosomal dominant - adults
Autosomal recessive - presents in neonates and is usually picked up on antenatal scans
How does ARPKD present?
1) Oligohydramnios
2) Cystic enlargement of the renal collecting ducts
3) Pulmonary hypoplasia
4) Potter syndrome
5) Congenital liver fibrosis
What is Potter syndrome?
What is it caused by?
A group of findings associated with a lack of amniotic fluid and kidney failure in an unborn infant.
Cause –> oligohydramnios
What are the features of Potter syndrome?
Characterised by dysmorphic features such as underdeveloped ear cartilage, low set ears, a flat nasal bridge and abnormalities of the skeleton.
What age does Wilm’s tumour typically affect?
<5 y/o
What is the most common presenting feature of a Wilm’s tumour?
Abdo mass
Are Wilms’ tumours unilateral or bilateral?
Unilateral (95%)
What is the most common site of metastases of a Wilm’s tumour?
Lung
What is the criteria for a very urgent referral (for an appointment within 48 hours) in regard to Wilms’ tumour?
1) palpable abdo mass
2) an unexplained enlarged abdominal organ
3) unexplained visible haematuria
What is the initial investigation in Wilms’ tumour?
US of abdomen
Management of Wilms’ tumour?
1) Nephrectomy: surgical excision of the tumour along with the affected kidney
2) Adjuvant treatment: chemo or radiotherapy
Incomplete immunisation against which 2 pathogens are a risk factor for CNS infection?
1) HiB
2) Strep. pneumoniae
When is a urine culture indicated in a febcon?
1) Complex seizure
or
2) <18m old
When should parents call an ambulance for a febrile seizure?
Lasts >5 mins
When should urgent referral for assessment by a paediatrician be made for a febrile seizure?
1) First presentation of febrile convulsion
2) Diagnostic uncertainty
3) Aged <18 months; signs of CNS infection may be more subtle in these children
4) Antibiotics have recently been taken, due to potential masking of symptoms of meningitis
1st & 2nd line mx of focal seizures?
1st –> lamotrigine or levetiracetam
2nd –> carbamazepine
What features are seen in juvenile myoclonic epilepsy?
(3)
1) generalised seizures, typically after waking/sleep deprivation
2) daytime absences
3) myoclonic seizures (may develop before generalised seizure)
When may myoclonic seizures occur in JME?
Just before generalised seizure occurs
What 2 medications does management of infantile spasms involve?
1) Prednisolone/ACTH
2) Vigabatrin
What is Lennox-Gastaut syndrome?
Features?
EEG finding?
Multiple seizure types.
Features:
- atypical absences, falls, jerks
- 90% moderate-severe mental handicap
EEG –> slow spike
Which epilepsy syndrome may a keto diet be beneficial in?
Lennox-Gastaut
What is benign rolandic epilepsy?
Features?
Most common in childhood, more common in males.
Features: paraesthesia (e.g. unilateral face), usually on waking up.
What epileptic syndrome may atonic seizures be indicative of?
Lennox Gastaut
When should an EEG be performed?
Perform an EEG after the second simple tonic-clonic seizure.
Children are allowed one simple seizure before being investigated for epilepsy.
Notable side effects of carbamazepine?
1) agranulocytosis
2) aplastic anaemia
3) P450 inducer
What is agranulocytosis?
Severe form of neutropenia
What is aplastic anaemia?
Pancytopenia of the peripheral blood (i.e., significant decreases in the numbers of RBCs, WBCs and platelets)
Notable side effects of phenytoin?
1) folate & vitamin D deficiency
2) megaloblastic anaemia (folate def)
3) osteomalacia (vit D def)
Notable side effects of lamotrigine?
1) SJS
2) Leukopenia
Notable side effects of ethosuximide?
1) night terrors
2) rashes
What are breath holding spells often divided into?
1) cyanotic breath holding spells
2) reflex anoxic seizures (i.e. pallid breath holding spells)
When do reflex anoxic seizures occur?
When the child is startled (vagus nerve sends strong signals to heart that cause it to stop beating)
When do cyanotic breath holding spells occur?
When the child is really upset, worked up and crying.
After letting out a long cry they stop breathing, become cyanotic and lose consciousness.
Within a minute they regain consciousness and start breathing.
What condition have breath holding spells been linked with?
Iron deficiency anaemia
What is the most common cause of 1ary headache in children?
Migraine without aura
From what age can sumatriptan be used in the acute management of migraine?
≥12 y/o –> nasal spray
Note - ORAL triptans are not currently licensed in people < 18 years
What are the 3 options for migraine prophylaxis in adukts?
1) propanolol
2) amitriptyline
3) topiramate
Features of an abdominal migraine?
Central abdo pain >1 hour
