Revision - HUS, Wilm's Tumour, FebCon & Epilepsy

Question 1

Give 3 causes of HUS

Answer 1

1) E. coli 0157

2) Pneumococcal infection

3) HIV

Question 2

What triad of features is seen in HUS?

Answer 2

1) Microangiopathic haemolytic anaemia

2) AKI

3) Thrombocytopenia

Question 3

What increases the risk of HUS in gastroenteritis?

Answer 3

Use of antibiotics & antimotility agents (e.g. loperamide)

Question 4

What causes thrombocytopenia in HUS?

Answer 4

The formation of blood clots consumes platelets, leading to thrombocytopenia.

Question 5

What is typically the first symptom of HUS?

Answer 5

Diarrhoea that turns bloody within 3 days

Question 6

Features of HUS?

Answer 6

• bloody diarrhoea
• abdo pain
• jaundice
• pallor
• fever
• lethargy
• oliguria
• haematuria
• HTN
• bruising
• confusion

Question 7

Management of HUS?

Answer 7

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

Question 8

Which type of PKD usually affects adults and which affects children?

Answer 8

Autosomal dominant - adults

Autosomal recessive - presents in neonates and is usually picked up on antenatal scans

Question 9

How does ARPKD present?

Answer 9

1) Oligohydramnios

2) Cystic enlargement of the renal collecting ducts

3) Pulmonary hypoplasia

4) Potter syndrome

5) Congenital liver fibrosis

Question 10

What is Potter syndrome?

What is it caused by?

Answer 10

A group of findings associated with a lack of amniotic fluid and kidney failure in an unborn infant.

Cause –> oligohydramnios

Question 11

What are the features of Potter syndrome?

Answer 11

Characterised by dysmorphic features such as underdeveloped ear cartilage, low set ears, a flat nasal bridge and abnormalities of the skeleton.

Question 12

What age does Wilm’s tumour typically affect?

Answer 12

<5 y/o

Question 13

What is the most common presenting feature of a Wilm’s tumour?

Answer 13

Abdo mass

Question 14

Are Wilms’ tumours unilateral or bilateral?

Answer 14

Unilateral (95%)

Question 15

What is the most common site of metastases of a Wilm’s tumour?

Answer 15

Lung

Question 16

What is the criteria for a very urgent referral (for an appointment within 48 hours) in regard to Wilms’ tumour?

Answer 16

1) palpable abdo mass

2) an unexplained enlarged abdominal organ

3) unexplained visible haematuria

Question 17

What is the initial investigation in Wilms’ tumour?

Answer 17

US of abdomen

Question 18

Management of Wilms’ tumour?

Answer 18

1) Nephrectomy: surgical excision of the tumour along with the affected kidney

2) Adjuvant treatment: chemo or radiotherapy

Question 19

Incomplete immunisation against which 2 pathogens are a risk factor for CNS infection?

Answer 19

1) HiB

2) Strep. pneumoniae

Question 20

When is a urine culture indicated in a febcon?

Answer 20

1) Complex seizure

or

2) <18m old

Question 21

When should parents call an ambulance for a febrile seizure?

Answer 21

Lasts >5 mins

Question 22

When should urgent referral for assessment by a paediatrician be made for a febrile seizure?

Answer 22

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

Question 23

1st & 2nd line mx of focal seizures?

Answer 23

1st –> lamotrigine or levetiracetam

2nd –> carbamazepine

Question 24

What features are seen in juvenile myoclonic epilepsy?

(3)

Answer 24

1) generalised seizures, typically after waking/sleep deprivation

2) daytime absences

3) myoclonic seizures (may develop before generalised seizure)

Question 25

When may myoclonic seizures occur in JME?

Answer 25

Just before generalised seizure occurs

Question 26

What 2 medications does management of infantile spasms involve?

Answer 26

1) Prednisolone/ACTH 2) Vigabatrin

Question 27

What is Lennox-Gastaut syndrome? Features? EEG finding?

Answer 27

Multiple seizure types. Features: - atypical absences, falls, jerks - 90% moderate-severe mental handicap EEG –> slow spike

Question 28

Which epilepsy syndrome may a keto diet be beneficial in?

Answer 28

Lennox-Gastaut

Question 29

What is benign rolandic epilepsy? Features?

Answer 29

Most common in childhood, more common in males. Features: paraesthesia (e.g. unilateral face), usually on waking up.

Question 30

What epileptic syndrome may atonic seizures be indicative of?

Answer 30

Lennox Gastaut

Question 31

When should an EEG be performed?

Answer 31

Perform an EEG after the second simple tonic-clonic seizure. Children are allowed one simple seizure before being investigated for epilepsy.

Question 32

Notable side effects of carbamazepine?

Answer 32

1) agranulocytosis 2) aplastic anaemia 3) P450 inducer

Question 33

What is agranulocytosis?

Answer 33

Severe form of neutropenia

Question 34

What is aplastic anaemia?

Answer 34

Pancytopenia of the peripheral blood (i.e., significant decreases in the numbers of RBCs, WBCs and platelets)

Question 35

Notable side effects of phenytoin?

Answer 35

1) folate & vitamin D deficiency 2) megaloblastic anaemia (folate def) 3) osteomalacia (vit D def)

Question 36

Notable side effects of lamotrigine?

Answer 36

1) SJS 2) Leukopenia

Question 37

Notable side effects of ethosuximide?

Answer 37

1) night terrors 2) rashes

Question 38

What are breath holding spells often divided into?

Answer 38

1) cyanotic breath holding spells 2) reflex anoxic seizures (i.e. pallid breath holding spells)

Question 39

When do reflex anoxic seizures occur?

Answer 39

When the child is startled (vagus nerve sends strong signals to heart that cause it to stop beating)

Question 40

When do cyanotic breath holding spells occur?

Answer 40

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.

Question 41

What condition have breath holding spells been linked with?

Answer 41

Iron deficiency anaemia

Question 42

What is the most common cause of 1ary headache in children?

Answer 42

Migraine without aura

Question 43

From what age can sumatriptan be used in the acute management of migraine?

Answer 43

≥12 y/o –> nasal spray Note - ORAL triptans are not currently licensed in people < 18 years

Question 44

What are the 3 options for migraine prophylaxis in adukts?

Answer 44

1) propanolol 2) amitriptyline 3) topiramate

Question 45

Features of an abdominal migraine?

Answer 45

Central abdo pain >1 hour