Immunology, Development And Neurology Flashcards

1
Q

What is the definition of ADHD ?

A

A condition incorporating features relating to inattention and/or hyperactivity/ impulsivity that are persistent.

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2
Q

What are the inattention features of ADHD ?

A

Does not follow through on instructions
Reluctant to engage in mentally-intense tasks
Easily distracted
Finds it difficult to sustain tasks
Forgetful

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3
Q

What are the hyperactive / impulse features of ADHD ?

A

Unable to play quietly
Talks excessively
Does not wait there turn
Interruptive and intrusive

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4
Q

What is the management of ADHD in children ?

A
  • 10 week watch and wait period to see if symptoms change
  • referral to secondary care
  • first line medication is methylphenidate
  • second line is lisdexamfetamine
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5
Q

What should be performed before starting ADHD medications ?

A

Baseline ECG as the medications are potentially cardio toxic.

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6
Q

What is ASD ?

A

Autism is a neurodevelopmental condition characterised by qualitative impairment in social interaction and communication as well as repetitive stereotyped behaviour, interests and activities.

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7
Q

What are some clinical features of ASD ?

A
  • Playing alone and uninterested in being with other children
  • stereotyped and repetitive motor mannerisms
  • inflexible routine
  • associated with intellectual impairment
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8
Q

What is the goal of the management of ASD ?

A
  • Learning and development, improved social skills and improved communication
  • decreased disability and comorbidity
  • aid to families
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9
Q

What are some therapies for ASD ?

A

Applied behavioural analysis
ASD preschool program
Structured teaching method
Family support and counselling

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10
Q

What is the definition of cerebral palsy ?

A

It may be defined as a disorder of movement and posture due to a non-progressive lesion of the motor pathways in the developing brain.

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11
Q

What are the antenatal causes of cerebral palsy ?

A

Cerebral malformation
Congenital infection - rubella, toxoplasmosis, CMV

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12
Q

What are the intrapartum causes of cerebral palsy ?

A

Birth asphyxia
Trauma

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13
Q

What are the postpartum causes of cerebral palsy ?

A

Intraventricular haemorrhage
Meningitis
Head trauma

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14
Q

What are some possible manifestations of cerebral palsy ?

A

Abnormal tone early infancy
Delayed motor milestones
Abnormal gait
Feeding difficulties

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15
Q

What is the most common classification of cerebral palsy ?

A

Spastic - increased tone resulting from damage to upper motor neurones

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16
Q

What is monoplegia ?

A

One leg affected

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17
Q

What is the hemiplegic ?

A

One side of the body is affected

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18
Q

what is diplegia ?

A

Four limbs are affected but mostly the legs

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19
Q

what is quadriplegia ?

A

Four limbs are affected more severely
Often with seizures, speech disturbance and other impairments

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20
Q

What are the signs and symptoms of cerebral palsy ?

A

Failure to meet milestones
Increased and decreased tone
Hand preferences below 18 months
Problems with coordination, speech or walking
Learning difficulties

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21
Q

Where is the lesion if someone has a hemiplegic gait ?

A

Upper motor neurone lesion

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22
Q

Where is the lesion if someone has an ataxic gait ?

A

Cerebellar lesion

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23
Q

Where is the lesion if someone has a high stepping gait ?

A

Foot drop
Lower motor neurone lesion

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24
Q

Where is the lesion if someone has a waddling gait ?

A

Pelvic muscle weakness due to myopathy

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25
Q

What is the gait exhibited in cerebral palsy ?

A

Hemiplegic or diplegic

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26
Q

What are signs of a upper motor neurone lesion ?

A

Muscle bulk preserved
Hypertonia
Power slightly reduced
Brisk reflexes

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27
Q

What are some complications and associated conditions of cerebral palsy ?

A

Learning disability
Epilepsy
Kyphoscoliosis
Muscle contractures
Hearing and visual impairment
GORD

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28
Q

What is the management of cerebral palsy ?

A

Life long conditions
Physiotherapy - stretch and strengthen muscles, maximise function and prevent muscle contractures
Occupational therapy - manage everyday activities
Speech and language therapy - speech and swallowing

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29
Q

What is the recommended length of time for breast feeding ?

A

WHO recommends exclusive breastfeeding for the first 6 months of life

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30
Q

What are the issues with breastfeeding ?

A

Poor milk supply
Difficulty latching
Discomfort or pain

Can all lead to inadequate nutrition for the baby

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31
Q

What are some benefits of breastfeeding ?

A

Contains antibodies that can help protect the neonate against infection
Lower infections in the neonate period
Better cognitive development
Reduced risk of sudden infant death syndrome

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32
Q

What can breastfeeding reduce the risk of in mothers ?

A

Breast cancer
Ovarian cancer

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33
Q

How much milk should a baby receive perKg of body weight ?

A

150ml

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34
Q

When is it acceptable for babies to lose up to 10% of their body weight ?

A

Up to day 5 but should return to birth weight by day 10

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35
Q

What are the most common causes of excessive weight loss in babies ?

A

Dehydration due to underfeeding

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36
Q

What is weaning ?

A

The gradual transition from milk to normal food.

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37
Q

When does weaning start ?

A

Around 6 months of age

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38
Q

What is a growth chart ?

A

Used to plot a child’s weight, height and head circumference against the normal distribution for their age and gender.

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39
Q

What are the phases of growth ?

A

First 2 years - rapid growth driven by nutritional factors
From year 2 to puberty - steady slow growth
During Puberty - rapid growth spurt driven by sex hormones

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40
Q

What is overweight and obese defined as in percentile in children

A

85th - overweight
95th - obese

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41
Q

What is the biggest immediate effect of obesity in children ?

A

Bullying

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42
Q

What are obese children at a higher risk of developing ?

A

Impaired glucose tolerance
T2 diabetes
CVD
Arthritis
Certain types of cancer

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43
Q

What is failure to thrive ?

A

Poor physical growth and developmental in a child

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44
Q

What is failure to thrive ?

A

Refers to poor physical growth and development in a child

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45
Q

What are some broad causes of failure to thrive ?

A

Inadequate nutritional intake
Difficulty feeding
Malabsorption
Increased energy requirements
Inability to process nutrition

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46
Q

What can cause inadequate nutritional intake causing failure to thrive ?

A

Maternal malabsorption if breastfeeding
Iron deficiency anaemia
Family or parental problems
Neglect
Availability of food

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47
Q

What can cause difficulty feeding causing failure to thrive ?

A

Poor suck - cerebral palsy
Cleft lip or palate
Pyloric stenosis

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48
Q

What can cause malabsorption causing failure to thrive ?

A

Cystic fibrosis
Coeliac disease
Cow’s milk intolerance
Chronic diarrhoea
IBD

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49
Q

What can cause increased energy requirements causing failure to thrive ?

A

Hyperthyroidism
Chronic disease - congenital heart disease and cystic fibrosis
Malignancy
Chronic infections - HIV or immunodeficiency

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50
Q

What can cause inability to process nutrients properly causing failure to thrive ?

A

Inborn errors of metabolism
T1 diabetes

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51
Q

What are the key areas to assess in a child failing to thrive ?

A

Pregnancy, birth, developmental and social history
Feeding or eating history
Observe feeding
Mums physical and mental health
Parent-child interactions
Height, weight and BMI and plotting these on a growth chart
Calculate mid-parental height centile

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52
Q

What are some investigations that should be performed if a child is failing to thrive ?

A

Urine dipstick - UTI
Coeliac screen

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53
Q

What is the management for a child failing to thrive ?

A

If breastfeeding is the cause - support for mothers can be given as well as supplemental milk
Encourage regular structured mealtimes and snacks
Reduce milk consumption to improve appetite for other foods
Review by a dietician
Energy dense foods

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54
Q

What is a short stature ?

A

As a height more than 2 standard deviations below the average for their age and sex

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55
Q

What are some causes of short stature ?

A

Familial short stature
Constitutional delay in growth and development
Malnutrition
Chronic diseases - coeliac, IBD,
Endocrine disorders - hypothyroidism
Genetic conditions - Down syndrome
Skeletal dysplasia’s - achondroplasia

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56
Q

What is constitutional delay in growth and puberty ?

A

It is considered a variation on normal development.
It leads to a short stature in childhood when compared with peers but normal height in adulthood.
Puberty is delayed and their growth spurt lasts longer

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57
Q

What are some milestones of gross motor function ?

A

4 months - support their own head
9 months - sit unsupported, crawling
12 months - stand
15 months - walk unaided
2 years - run and kick a ball

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58
Q

What are some milestones of fine motor function ?

A

6 months - palmar grasp
9 months - scissor grasp of objects
12 months - pincer grasp

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59
Q

What are some milestones of expressive language ?

A

3 months - cooing noise
9 months - talking but not recognisable
12 months - dada
18 months - 5 to 10 words
3 years - using basic sentences

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60
Q

What are some milestones of receptive language ?

A

3 months - recognises parents and familiar voices
6 months - responds to tone of voice
9 months - listens to speech
18 months - understands nouns
2 years - understands verbs
3 years - understands adjectives

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61
Q

What are some milestones for personal and social skills ?

A

6 weeks - smile
3 months - communicates pleasure
12 months - engages with others by pointing and handing objects
2 years -waving at strangers
3 years - play with other children , bowel control

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62
Q

What are some red flags in terms of developmental milestones ?

A

Not able to hold an object at 5 months
Not sitting unsupported at 12 months
Not standing independently at 18 months
Not walking independently at 2 years
No words at 18 months
No interest in others at 18 months

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63
Q

What is global developmental delay ?

A

It refers to a child displaying slow development in all developmental domains. This could indicate an underlying diagnosis such as :
- Down’s syndrome
- fragile X syndrome
- foetal alcohol syndrome

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64
Q

What are some potential causes of gross motor delay ?

A

Cerebral palsy
Ataxia
Myopathy
Spina bifida
Visual impairment

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65
Q

What are some potential causes of fine motor delay ?

A

Dyspraxia
Cerebral palsy
Muscular dystrophy
Visual impairment

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66
Q

What are some potential causes of language delay ?

A

Hearing impairment
Learning disability
Neglect
Autism
Cerebral palsy

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67
Q

What are some potential causes of personal and social delay ?

A

Emotional and social neglect
Parenting issues
Autism

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68
Q

What is dyslexia ?

A

A difficulty in reading, writing and spelling

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69
Q

What is dysgraphia ?

A

A specific difficulty in writing

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70
Q

What is dyspraxia ?

A

Also known as developmental co-ordination disorder.

Refers to a specific type of difficulty in physical co-ordination.
It presents with delayed gross and fine motor skills and a child that appears clumsy.

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71
Q

What is the severity of learning disabilities based on and what are the classes ?

A

IQ
55-70 = mild
40-55 = moderate
25-40 = severe
Under 25 = profound

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72
Q

What are the causes of learning difficulties ?

A

No clear cause
Family history, abuse, neglect and psychological trauma increases the risk

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73
Q

What conditions are associated with learning difficulties ?

A

Genetic disorders such as Down’s syndrome
Antenatal problems - foetal alcohol syndrome and maternal chicken pox
Problems in early childhood - meningitis
Autism
Epilepsy

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74
Q

What must a patient have to demonstrate capacity ?

A

Understand the decision
Retain the information
Weigh up the options and the implications of choosing each option
Communicate their decision

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75
Q

When does puberty normally take place in boys and girls ?

A

8-14 in girls
9-15 in boys

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76
Q

What are the stages of puberty in girls ?

A

Development of breast buds
Then pubic hair
Lastly menstrual periods

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77
Q

What are the stages of puberty in boys ?

A

Starts with enlargement of testicles
Then penis
Gradual darkening of the scrotum
Development of pubic hair and deepening of the voice

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78
Q

What is hypogonadotropic hypogonadism ?

A

There is a deficiency of LH and FSH leading to a deficiency of the sex hormones testosterone and oestrogen. This is usually due to an abnormal functioning of the hypothalamus or pituitary gland.

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79
Q

What causes hypogonadotropic hypogonadism ?

A

Previous damage to the hypothalamus or pituitary - radiotherapy or surgery
Growth hormone deficiency
Hypothyroidism
Hyperprolactinaemia
Excessive exercise or dieting
Kallman syndrome

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80
Q

What is hypergonadotropic hypogonadism ?

A

This is where the gonads fail to respond to stimulation from the gonadotropins ( LH and FSH ).
There is no negative feedback from the sex hormones causing high levels of LH and FSH.

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81
Q

What is Kallman syndrome ?

A

A genetic condition causing hypogonadotropic hypogonadism resulting in failure to start puberty. It is associated with a reduced or absent sense of smell ( anosmia ).

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82
Q

What are the types of abuse ?

A

Physical
Emotional
Sexual
Neglect
Financial
Identity

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83
Q

What are some risk factors for abuse ?

A

Domestic violence
Previously abused parent
Mental health problems
Emotional volatility in the household
Disability in the child
Alcohol misuse
Substance misuse

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84
Q

What are some possible signs of abuse ?

A

Change in behaviour or extreme emotional states
Dissociative disorders ( feeling separated from their thoughts or identity )
Bullying, self harm or suicidal behaviours
Unusually sexualised behaviours
Unusual behaviour during examination
Poor hygiene

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85
Q

What must a child under 16 have to make a decision about their treatment ?

A

Gillick competence

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86
Q

What is Gillick competence ?

A

Refers to a judgement about whether the understanding and intelligence of the child is sufficient to consent to treatment.
Needs to be assessed on a decision by decision basis.

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87
Q

What are some typical symptoms of depression ?

A

Low mood
Anhedonia
Low energy
Anxiety
Irritability
Hopelessness about future
Poor appetite
Poor concentration

88
Q

What are some important psychosocial contributors to depression in adolescents ?

A

Potential triggers
Home environment
Family relationships
Friendships
Sexual relationships
School situations
Bullying
Drugs and alcohol
Self harm
Family history

89
Q

How is mild depression managed in children ?

A

Watchful waiting and advice about healthy habits
Follow up in 2 weeks

90
Q

How is moderate - severe depression managed?

A

Full assessment
Psychological therapy - CBT, family therapy, interpersonal therapy
Fluoxetine - first line
Sertraline and citalopram - 2nd

91
Q

When is admission required for a child with depression ?

A

High risk of self harm, suicide or self neglect
Immediate safeguarding issue

92
Q

What is GAD?

A

Excessive and disproportionate anxiety and worry that negatively impacts the persons everyday activity

93
Q

What is an allergy ?

A

An umbrella term fro hypersensitivity of the immune system to allergens.

94
Q

What are allergens ?

A

They are proteins that the immune system recognises as foreign and potentially harmful, leading to an allergic immune response.

95
Q

What is atopy ?

A

A term used to describe a predisposition to having hypersensitivity reactions to allergens.
( eczema, asthma, hay fever, allergic rhinitis and food allergies ).

96
Q

What are some conditions that are due to hypersensitivity reactions ?

A

Asthma
Atopic eczema
Allergic rhinitis
Hay fever
Food allergies
Animal allergies

97
Q

What is type 1 hypersensitivity reaction ?

A

IgE antibodies to a specific allergen trigger mast cells and basophils to release histamines and cytokines. This causes an immediate reaction.
( typical food allergy )

98
Q

What is type 2 hypersensitivity reaction ?

A

IgG and IgM antibodies react to an allergen and activate the complement system, leading to direct damage to the local cells.
( haemolytic disease of the newborn )

99
Q

What is type 3 hypersensitivity reaction ?

A

Immune complexes accumulate and cause damage to local tissues
( SLE, RA

100
Q

What is type 4 hypersensitivity reaction ?

A

Cell mediated hypersensitivity reactions caused by T lymphocytes. T cells are inappropriately activated causing inflammation and damage to local tissues ( organ transplant rejection or contact dermatitis )

101
Q

What are some specific questions to ask when taking a history of an allergy ?

A

Timing after exposure to the allergen
Previous and subsequent exposure and reaction to the allergen.
Symptoms of a rash, swelling, breathing difficulty, wheeze and cough
Previous personal and family history of atopic conditions or allergies

102
Q

What investigations are there for allergies and what is gold standard?

A

Skin prick testing
RAST testing
Food challenge testing - gold standard

103
Q

How is skin testing for allergies performed ?

A

A patch of skin is selected.
Strategic allergen solutions are selected.
A drop of each allergen is placed at mark3d points along the patch of skin along with a water and histamine control.
A needle is used to put a tiny break in the skin

104
Q

What is patch testing ?

A

Most helpful in determining an allergic contact dermatitis in response to a specific allergen. This could be for latex, perfumes, cosmetics or plants.
A patch is placed on the patients skin and after 2-3 days the skin is reassessed.

105
Q

What is RAST testing ?

A

RAST testing measures the total and allergen specific IgE quantities in the patients blood sample. Patients with atopic conditions such as eczema and asthma will come back positive.

106
Q

What is the food challenge ?

A

A food challenge should be performed in a specialised unit with very close monitoring. The child is gradually given increasing amounts of the allergen to assess the reaction.

107
Q

What is the management of allergies ?

A

Establish the correct allergen
Avoidance
Prophylactic antihistamines
Auto-injector

108
Q

What are febrile convulsions ?

A

A type of seizure that occurs in children with a high fever. They are not caused by epilepsy or other neurological pathology, such as meningitis. Only occur between ages of 6 months and 5 years.

109
Q

What is a simple febrile convulsion ?

A

Generalised tonic clonic seizures. They last less than 15 minutes and only once during a single febrile illness.

110
Q

What are some differentials for febrile convulsions ?

A

Epilepsy
Meningitis, encephalitis
Intracranial space occupying lesion
Syncopal episode
Electrolyte imbalance
Trauma

111
Q

What is the typical presentation of febrile convulsion ?

A

18 months old presenting with a 2-5 minute tonic clonic seizure during a high fever.
Usually caused by an underlying viral illness or bacterial infection.

112
Q

What is the management of febrile convulsions ?

A

Paracetamol and ibuprofen
Give advice for further episodes :
- stay with child
- put child in safe place
- place in recovery position
- call an ambulance if the seizure lasts more than 5 minutes

113
Q

What is allergic rhinitis ?

A

A condition caused by an IgE - mediated type 1 hypersensitivity reaction. Environmental allergens cause an allergic inflammatory response in the nasal mucosa.

114
Q

What are the types of allergic rhinitis ?

A

Seasonal - hay fever
Perennial - house dust mite allergy
Occupational - school or work environment

115
Q

What is the presentation of allergic rhinitis ?

A

Runny, blocked and itchy nose
Sneezing
Itchy, red and swollen eyes
Associated with family history

116
Q

What are the triggers of allergic rhinitis ?

A

Tree pollen or grass
House dust mites
Pets
Mould

117
Q

What is the management of allergic rhinitis ?

A

Avoid the trigger
Oral antihistamines -
- non sedating such as cetirizine
-sedating such as Chlorphenamine
Nasal corticosteroids

118
Q

What is cow’s milk allergy ?

A

A condition typically affecting infants and young children under 3 years old.
It involves hypersensitivity to the protein in cow’s milk and can either be IgE or non-IgE mediated.

119
Q

How does cow’s milk allergy present ?

A

Bloating and wind
Abdominal pain
Diarrhoea
Vomiting
Urticaria
Cough or wheeze
Sneezing

120
Q

What is the management of cow’s milk allergy ?

A

Avoid cow’s milk
Replace formula with hydrolysed formulas

121
Q

What are some signs that a child should see a specialist if having recurrent infections ?

A

Chronic diarrhoea since infancy
Failure to thrive
Appearing unusually well with a sever infection
Unusual or persistent infections such as cytomegalovirus, candidiasis and pneumocystitis jiroveci

122
Q

What investigations should be performed if a child has recurrent infections ?

A

FBC - low neutrophils suggest phagocyte disorder, low lymphocytes suggest T cell disorder
Immunoglobulins - abnormalities can suggest B cell disorder
Complement proteins
Antibody responses to vaccines, pneumococcal and Haemophilus vaccines
HIV test
CXR
Sweat test for CF
CT chest for bronchiectasis

123
Q

What is severe combined immunodeficiency ?

A

Most severe condition causing immunodeficiency
It is a syndrome caused by a number of different genetic disorders that result in absent or dysfunctioning T and B cells

124
Q

When does SCID present ?

A

In the first few months of life

125
Q

How does SCID present ?

A

Persistent severe diarrhoea
Failure to thrive
Opportunistic infections - pneumocystis jiroveci and cytomegalovirus
Unwell after live vaccinations
Omenn syndrome

126
Q

What are some causes of SCID ?

A

Mutations in the common gamma chain on X chromosome that codes for interleukin receptors on T and B cells. This is x linked recessive inheritance.
Other gene mutations - JAC3 gene mutations

127
Q

What is omenn syndrome ?

A

A rare cause of SCID. It is a result of a mutation in the RAG that codes for proteins in T and B cells.
Autosomal recessive

128
Q

What are some classic features of Omenn syndrome ?

A

A red scaly dry rash
Hair loss
Diarrhoea
Failure to thrive
Lymphadenopathy
Hepatosplenomegaly

129
Q

What is the management of SCID ?

A

Fatal unless treated
Specialist immunology centre
Management involves immunoglobulin therapy
Avoid live vaccines
Haematopoietic stem cell transplantation

130
Q

How do abnormal B cells lead to recurrent infections ?

A

B cells are responsible for producing antibodies. Abnormal b cells lead to a deficiency in immunoglobulins called hypogammaglobulinemia. This leads the patient more susceptible to recurrent infections.

131
Q

What is selective immunoglobulin A deficiency ?

A

Patients have low levels of IgA and normal IgG and IgM.
Mild immunodeficiency where patients are often asymptomatic
Often have recurrent mucous membrane infections - LRTI and autoimmune conditions

132
Q

Where is IgA present and what does it protect against ?

A

IgA is present in secretions of the mucous membranes such as saliva, resp tract secretions, tears and sweat.
IgA protects against opportunistic infections of these mucous membranes.

133
Q

What is common variable immunodeficiency ?

A

It is caused by a genetic mutation in the genes coding for components of B cells. The result is a deficiency in IgG and IgA with or without a deficiency in IgM. This leads to recurrent resp tract infections.
Inability to develop immunity to infections or vaccinations.

134
Q

What other disorders are people with common variable immunodeficiency prone to ?

A

RA
Cancers such as non-Hodgkin’s lymphoma

135
Q

What is the management of Common Variable Immunodeficiency ?

A

Regular immunoglobulin infusions and treating infections and complications

136
Q

What is X linked agammaglobulinaemia ?

A

Also known as bruton’s agammaglobulinaemia
X linked recessive
Results in abnormal B cell development and causes deficiency in all immunoglobulins

137
Q

What is DiGeorge syndrome ?

A

Results from a micro deletion in a portion of chromosome 22 that leads to a developmental defect in the third pharyngeal pouch and third bronchial cleft. One of the consequences is that the thymus doesn’t fully develop. This causes inability to create functional T cells.

138
Q

What are some features of DiGeorge syndrome ?

A

Congenital heart disease
Abnormal facies ( characteristic facial appearance )
Thymus gland incompletely developed
Hypoparathyroidism causing hypocalcaemia
22nd chromosome affected

139
Q

What is the importance of complement proteins in the immune system ?

A

They deal with encapsulated organism -
Haemophilus influenza B
Streptococcus pneumonia
Neisseria meningitidis

140
Q

What are complement deficiencies associated with ?

A

Immune complex disorders - SLE

141
Q

What is syncope ?

A

The term used to describe the event of temporarily losing consciousness due to a disruption of blood flow to the brain often leading to a fall.

142
Q

What causes a vasovagal episode ?

A

Caused by a problem with the autonomic nervous system regulating blood flow to the brain.
When the vagus nerve receives a strong stimulus it can stimulate the parasympathetic nervous system. This causes relaxation of blood vessels delivering blood to the brain leading to hypoperfusion

143
Q

What is the feeling of prodrome ?

A

Hot or clammy
Sweaty
Heavy
Dizzy or lightheaded
Blurry vision
Headache

144
Q

What are some causes of primary syncope ?

A

Dehydration
Missed meals
Extended standing in a warm environment
Stimuli such as sudden surprise, pain

145
Q

What are some causes of secondary syncope ?

A

Hypoglycaemia
Dehydration
Anaemia
Infection
Anaphylaxis
Valvular heart disease

146
Q

What are some features that would indicate syncope over a seizure ?

A

Prolonged upright position before event
Lightheaded before event
Sweating before event
Blurring or clouding of vision
Reduced tone during episode
Quick return of consciousness
No prolonged post ictal period

147
Q

What are some features that would indicate seizure over a syncope ?

A

Epilepsy aura ( smells, tastes ) before event
Head turning or abnormal limb movement
Tonic clonic activity
Tongue biting
Cyanosis
Lasts longer than 5 minutes
Prolonged post ictal period

148
Q

What are some investigations performed for syncope ?

A

ECG
24 hour ECG
Echocardiogram
Bloods - FBC, U&E’s
Blood glucose

149
Q

What is the treatment of a vasovagal episode ?

A

Avoid dehydration
Avoid missing meals
Avoid standing still for long periods of time
When experiencing prodrome sit down

150
Q

What is a seizure ?

A

Transient episodes of abnormal electrical activity in the brain.

151
Q

What is a generalised tonic clonic seizure ?

A

Loss of consciousness and tonic ( muscle tensing ) and clonic ( muscle jerking ) movements.
Typically tonic precedes clonic.
Associated signs - tongue biting, incontinence, groaning and irregular breathing

152
Q

What is the management of tonic clonic seizures ?

A

First line - sodium valproate
Second line - Lamotrigine or carbamazepine

153
Q

What are focal seizures ?

A

Start in the temporal lobes
Affect hearing, speech, memory and emotions

154
Q

How do focal seizures present ?

A

Hallucinations
Memory flashbacks
Deja vu
Doing strange things on autopilot

155
Q

What is the management for focal seizures ?

A

First line - carbamazepine or Lamotrigine
Second line - sodium valproate or Keppra

156
Q

What is an absence seizure ?

A

The patient becomes blank, stares into space and then abruptly returns to normal.
They are unresponsive in this period
Last 10-20 seconds

157
Q

What is the management of absence seizures ?

A

Sodium valproate or ethosuximide

158
Q

What is an atonic seizure ?

A

Drop attacks
Lapse in muscle tone
Usually last less than 3 minutes

159
Q

What may atonic seizures be indicative of ?

A

Lennox-Gastaut syndrome

160
Q

What is the management of atonic seizures ?

A

First line - sodium valproate
Second line - lamotrigine

161
Q

What is a myoclonic seizure ?

A

Sudden brief muscle contraction - sudden jump

162
Q

If a child is having seizures what investigations should be performed ?

A

EEG
MRI brain
ECG
Blood electrolytes
Blood glucose
Blood cultures, urine cultures and LP

163
Q

What is some general advice for patients and families who are having seizures ?

A

Take showers rather than baths
Be cautious when swimming, with heights and traffic
Be cautious when carrying heavy, hot or electrical equipment

164
Q

How does sodium valproate work ?

A

Increases the activity of GABA which has a relaxing effect on the brain

165
Q

What are some side effects of sodium valproate ?

A

Teratogenic ( contraception advice )
Liver damage and hepatitis
Hair loss
Tremor

166
Q

What are some side effects of carbamazepine ?

A

Agranulocytosis
Aplastic anaemia
Induces P450 system so drug interactions

167
Q

What are some side effects of phenytoin ?

A

Folate and vitamin D deficiency
Megaloblastic anaemia
Osteomalacia

168
Q

What are some side effects of ethosuximide ?

A

Night tremors
Rashes

169
Q

What are some side effects of Lamotrigine ?

A

Stevens-Johnson syndrome
Leukopenia

170
Q

What is the immediate management of a seizure ?

A

Put the patient in a safe position
Place in recovery position
Put something soft under their head
Remove objects
Make note of start and end of seizure
Call an ambulance if longer than 5 minutes

171
Q

What is status epilepticus ?

A

A seizure lasting more than 5 minutes or 2 or more seizures without regaining consciousness in the interim.

172
Q

What is the management of status epilepticus ?

A

Secure the airway
Give high concentration o2
Assess cardiac and resp function
Check blood glucose levels
Gain IV access
Iv lorazepam - repeated after 10 minutes if seizure continues
Then keppra if continues after that

173
Q

What is a febrile convulsion ?

A

A type of seizure that occurs in children with a high fever
Occur in ages 6 months to 6 years

174
Q

What is a simple febrile convulsion ?

A

Generalised tonic clonic seizure
Lasts less than 15 minutes
Only occurs once during a single febrile illness

175
Q

What is a complex febrile convulsion ?

A

They consist of partial or focal seizure, last longer than 15 minutes or occur multiple times during the same febrile illness

176
Q

What are some differentials for febrile convulsions ?

A

Epilepsy
Meningitis, encephalitis
Intra-cranial space occupying lesion - brain tumour
Syncopal episode
Electrolyte abnormalities
Trauma

177
Q

What are some causes of headaches ?

A

Tension headaches
Migraines
ENT infection
Visual problems
Raised ICP
Brian tumours
Meningitis
Encephalitis

178
Q

What are the features of a tension headache ?

A

Mild ache across the forehead
Pain or pressure in a band-like pattern
Come on and resolve gradually
No visual changes or pulsating sensations
Symmetrical

179
Q

What are some triggers for a tension headache ?

A

Stress, fear, discomfort
Skipping meals
Dehydration
Infection

180
Q

What is the management of tension headaches ?

A

Reassurance
Analgesia
Dehydration
Reducing stress

181
Q

What are the differences between migraines and tension headaches?

A

unilateral
More severe
Throbbing in nature
Take longer to resolve

182
Q

What are migraines often associated with ?

A

Visual aura
Photophobia and photophobia
Nausea and vomiting
Abdominal pain

183
Q

What are the management options for migraines ?

A

Rest, fluids and low stimulus
Paracetamol
Ibuprofen
Sumatriptan
Antiemetics - Domperidone

184
Q

Who are more likely to develop abdominal migraines and how do they present ?

A

Children
Episodes of central abdo pain lasting more than 1 hour

185
Q

What is a squint ?

A

Misalignment of the eyes
Also known as strabismus

186
Q

ho

A
187
Q

How does a squint cause double vision ?

A

When the eyes are not aligned, the images on the retina do not match and the person will experience double vision

188
Q

How does a squint cause a lazy eye ?

A

Before the eyes fully establish their connection with the brain, the brain will cope with the misalignment by reducing the signal from the less dominant eye. This results in one eye becoming progressively more disconnected from the brain and over time the problem gets worse. This is called amblyopia.

189
Q

What is strabismus ?

A

The eyes are misaligned

190
Q

What is amblyopia ?

A

The affected eye becomes passive and has reduced function compared to the dominant one

191
Q

What is esotropia ?

A

Inward positioned squint ( affected eye towards the nose )

192
Q

What is exotropia ?

A

Outward positioned squint ( affected eye towards the ear )

193
Q

What is hypertropia ?

A

Upward moving affected eye

194
Q

What is hypotropia ?

A

Downward moving affected eye

195
Q

What are some causes of a squint ?

A

Idiopathic
Hydrocephalus
Space occupying lesion - retinoblastoma
Trauma

196
Q

What examinations should be performed for a squint ?

A

General inspection
Eye movements
Fundoscopy - rule out retinoblastoma, cataracts
Visual acuity

197
Q

What is Hirschberg’s test ?

A

Shine a pen torch at the patient from 1 meter away.
When they look at it observe the reflection of the light source on the cornea. The reflection should be central and symmetrical.
Deviation from the centre indicates squint

198
Q

What is the cover test ?

A

Cover one eye and ask the patient to focus on an object in front of them.
Move the cover across to the opposite eye and watch the movement of the previously covered eye.

199
Q

What is the management of a squint ?

A

Treatment needs to be started before 8 years old
Occlusive patch - cover stronger eye
Atropine drops - in good eye
Managed by ophthalmologist

200
Q

What is hydrocephalus ?

A

Describes cerebrospinal fluid building up abnormally within the brain. This is due to either overproduction of CSF or a problem draining or absorbing CSF

201
Q

How does the CSF normally drain ?

A

There are 4 ventricles in the brain ( 2 lateral, the third and the fourth ).
They contain CSF. CSF is created by the choroid plexus and by the walls of the ventricles.
CSF is absorbed into the venous system by the arachnoid granulations.

202
Q

What are some congenital causes of hydrocephalus ?

A

Aqueductal stenosis - cerebral aqueduct that connects the third and fourth ventricle is narrowed. This causes a build up in the lateral and third ventricles.
Arachnoid cyst
Arnold-Chiari

203
Q

How do babies with hydrocephalus present ?

A

As the cranial bones aren’t fused yet the skull expands to fit the cranial contents - enlarged head
Bulging anterior fontanelle
Poor feeding and nausea
Poor tone
Sleepiness

204
Q

What is a ventriculoperitoneal shunt ?

A

Placing a VP shunt that drains CSF from the ventricles into another body cavity - usually peritoneal.
A small tube is placed through a small hole in the skull at the back of the head and into one of the ventricles.

205
Q

What are some complications of VP shunt ?

A

Infection
Blockage
Excessive drainage
Intraventricular haemorrhage
Outgrowing them

206
Q

What is plagiocephaly ?

A

Flattening of one area of the baby’s head

207
Q

What is brachycephaly ?

A

Flattening of the back of the head resulting in a short head from back to front

208
Q

What is the management of plagiocephaly and brachycephaly ?

A

Reassurance
Position the on the rounded side for sleep
Supervised tummy time
Using rolled towels as props

209
Q

What is muscular dystrophy ?

A

An umbrella term for genetic conditions that cause gradual weakening and wasting of muscles.

210
Q

What is the main muscular dystrophy ?

A

Duchennes muscular dystrophy

211
Q

What is Gower’s sign ?

A

To stand up from a lying position they go onto their hands and knees then push their hips up - downward dog - then put their hands on their knees and then walk their hands up their leg to stand up. Weak pelvic muscles - muscular dystrophy

212
Q

What is the inheritance of duchennes muscular dystrophy ?

A

X linked recessive

213
Q

What is the management for Duchennes muscular dystrophy ?

A

No curative treatment
Physio
Occupational therapy

214
Q

What is defective in Duchennes muscular dystrophy ?

A

Defective gene for dystrophin on the X chromosome

215
Q

What are some management options for duchennes muscular dystrophy ?

A

Oral steroids have been shown to slow the progression of muscle weakness by as much as 2 years
Creatine supplements

216
Q

What is spinal muscular atrophy ?

A

A rare autosomal recessive condition that causes progressive loss of motor neurones leading to progressive muscular weakness.
Affects lower motor neurones

217
Q

What are some lower motor neurone signs ?

A

Fasciculations
Reduced muscle bulk
Reduced tone
Reduced power
Reduced or absent reflexes