Paeds Flashcards

1
Q

What is Ipratropium (atrovent)?

A

Muscarinic antagonist used for bronchodilation

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2
Q

Differences in Bronchiolitis and Viral induced Wheeze?

A

Both present with cough, coryza, wheeze and possibly some respiratory distress

  1. Brochiolitis does not respond to inhalers, viral wheeze does
  2. Most children under the age of 1 will have bronchiolitis
  3. Resp distress in viral induced wheeze is caused by bronchospasm induced by the viral infection. In Bronciolitis it is caused by the large numbers of secretions in the airways.
  4. Viral induced wheeze will cause resp distress over a short space of time, bronchiolitis will cause a slow progression to respiratory distress
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3
Q

What is a macular rash, a vesicular rash, a papular rash and a pustular rash?

A

Macular:
- change in colour of the skin - normally associated with erythema

Vesicular:
- Clear fluid filled blisters

Papular:
- Solid raised lesion

Pustular:
- Pus filled blisters

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4
Q

What does coryzal mean?

A

Cold-like symptoms of the upper respiratory tract snotty inflamamtion of nasal passages

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5
Q

What is brittle bone disease?

A

Oteogenesis imperfecta

Group of genetic disorders affecting bone development (8 types)

Lack of type 1 collagen production

Types vary from mild symptoms to life threatening ones

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6
Q

What is Duchenne’s muscular dystrophy?

A

X-linked recessive disorder

Lack of protein dystrophin

Normally present at about 5 years old

Chair at about 8

Life expectancy is avg. 25 years

Progressive muscle weakness, often originally affecting proximal muscles of shoulder and thigh

Also affects smooth muscle - Respiratory failure is ultimate cause of death.

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7
Q

What is tuberous sclerosis?

A

Genetic condition that causes benign tumours to form, these are usually present from birth.

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8
Q

What is Montelukast?

A

Leukotriene receptor antagonist for treatment of asthma.

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9
Q

What are CPAP and BiPAP

A

Both create positive pressure to keep the airways open (usually used when sleeping e.g. in muscular dystrophy)

CPAP is continuous
BiPAP has two pressure settings - one for inhalation and one for exhalation.

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10
Q

What is kawasaki syndrome?

A

Rare Vasculitis

Range of symptoms, as described by a japanese doctor - it can be sub acute, acute and convalescent. Fever, red palms, strawberry tongue, lymphadenopathy

Treated with aspirin and antibodies

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11
Q

Triad of symptoms in autism?

A

Lack of:

  1. Social and emotional communication
  2. Imagination/flexibility of thought - activities and interests
  3. Social and emotional interaction
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12
Q

Types of symptoms in autism?

A

Social interaction:
- Appears unresponsive
- Absent/unusual eye contact and expressions
- Does not spontaneously share interest and enjoyment
- Does not make friends

Social communication:
- Unusual/repetitive language
- Delayed/impoverished language development
- Does not initiate/maintain

Activities & Interests:
- Pre-occupation with narrow interests
- Unusual/repetitive gestures & actions
- Rigid routines

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13
Q

Normal baby weight?

A

2.7-4.1kg

6-9lbs

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14
Q

Verbal development stages?

A

1 year - one word
2 years - phrases
3 years - sentences

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15
Q

Causes of clubbing?

A

Bronchiectasis (CF)
Fibronising alveolitis
cyanotic congenital heart disease
IBD

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16
Q

4 T’s in relation to diabetes?

A

Tired
Thin
Thirsty
Toilet

Fatigue
Weight loss
Polydipsia
Polyuria

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17
Q

Prevalence of childhood diabetes?

A

1 in 400 children

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18
Q

Main risk factors of children with diabetes at time of presentation?

A

Younger children

Fmaily Hx (1st degree relative)

Lower SES

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19
Q

Normal range of fasting blood glucose (for kids)

A

6.1-7.8

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20
Q

Brief description of DKA?

A

Essentially it’s a lack of insulin (why it’s type 1) this causes two main things:

  1. Increased release of glucose production from glycogen - spilling over into the urine, taking Na and K and extra water with it leading to polyuria, polydipsia and dehydration.
  2. Body uses fat as an alternative source of fuel - to protect the metabolically active brain. This forms ketones in large quantities which lowers the blood pH and induced metabolic acidosis.
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21
Q

What percentage O2 do you use for acutely unwell children?

A

100% - don’t fuck about.

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22
Q

Complications associated with DKA?

A

Hyperglycaemia

Hypokalaemia

Cerebral oedema

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23
Q

DKA treatment?

A

SLOW insulin infusion
Fluid resus - Normal saline w/ K+

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24
Q

Treatment of cerebral oedema?

A

Sit child up
High conc saline
Intubation

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25
Signs of Cerebral oedema?
Biggest one - headache Tachycardia/HTN Also: - Nausea - Vomiting - Reduced consciousness
26
DO you give child with DKA water?
No - will most likely just vomit it up and this increases risk of aspiration - may even put in NG tube.
27
What is Alport syndrome?
Genetic condition affecting children, rare. Characterised by glomerulonephritis, end-stage kidney disease and hearing loss. Type IV collagen is affected.
28
Investigations in children with possible epilepsy?
EEG Bloods - magnesium ECG - rule out cardiac cause e.g. long-QT syndrome Brain imagining
29
Two types of absence epilepsy?
Childhood absence epilepsy - short fits, grow out if Juvenile absence epilepsy - longer fits, probably won't grow out of.
30
Types of epileptic seizures in childhood/generally?
Generalised: Absence - transient loss of consciousness, with abrupt onset and termination, normally unaccompanied by motor phenomena apart from some increase in muscle tone and eyelid flickering. Myoclonic seizures - Repetitive jerking movements of the limbs neck or trunk Tonic seizures - generalised increase in tone Tonic-Clonic - classic symptoms of a rigid tonic phase followed by rhythmic jerking movements, may also be accompanied by loss of continence, followed by deep sleep. Atonic seizures - jerk followed by transient loss of muscle tone. Focal - Frontal - motor - Temporal - auditory, smell or taste - Occipital - visual - Parietal - altered sensation (contralateral)
31
In what type of infection do lymphocytes fall and neutrophils rise?
Bacterial.
32
What are some of the possible causes of abdo masses in CF children?
Fibrosing colonopathy - right colon (RIF) Hepatosplenomegaly Constipation - LIF Gastrostomy tube.
33
What is hypospadias?
Urethral orifice is not in the usual position - different grades.
34
What is rhesus incompatibility and how does it affect the neonate?
Antibodies to rhesus D antigens have developed. Mother is Rh Negative and baby is positive then the mother may produce antibodies against the babies RhD antigens (on RBCs). Has to be second baby.
35
How many babies become jaundiced? What causes this around 24hrs?
Roughly 50% of babies are visually jaundiced. Prehepatic - excessive breakdown of red cells (haemolysis) Hepatic - abnormality in liver function (neonatal hepatitis) Posthepatic - absent or bile duct atresia, causes conjugated hyperbilirubinaemia Why is it important? Unconjugated bilirubin deposited in the brain (basal ganglia and brainstem nuclei) may cause irreversible neurological damage - kernicterus Normal physiological jaundice (>24hrs - weeks): - Marked physiological release of haemoglobin from the breakdown of red cells due to high haemoglobin conc at brith - Liver does not function as well. <24hrs may also be a sign of another disorder: - Haemolytic anaemia - Infection - Inborn error of metabolism - Liver disease
36
Haemolytic disorders of the newborn?
Rhesus haemolytic disease - Antibodies to rhesus D antigens have developed. Now mostly treated antenatally - low haemoglobin, hepatosplenomegaly, jaundice. Coombs test is positive. ABO incompatibility - Some group O women have IgG anti-A-Haemolysin in their blood, bad for group A infants. Group B infants also at risk. Hepatosplenomegaly is not usually present. Coombs test (detects antibody on red cells) is positive. G6PD (gluc. 6 dehydrogenase deficiency. - mainly in non-caucasian backgrounds. Mostly affects males Spherocytosis - Rare genetic disorder, Family history is present.
37
At what time of onset is jaundice worrying?
Before 24hrs - haemolytic disorder or infection. After 2 weeks - breast milk jaundice, biliary atresia
38
Signs of respiratory distress in the infant?
Head bobbing Nasal flaring Grunting Recession (4 types) Tachopnoea (>60 RR) Cyanosis
39
Why do you feed small quantities slowly and early to neonates?
To avoid Necrotising enterocolitis (NEC)
40
Normal neonatal obs?
HR <170 <60 RR BP - depends on gestation and age Sys: ranges from 48-72 roughly Dia: ranges from 25-50 Mean - above corrected gestation is normal
41
What is necrotizing enterocolitis?
Bowel becomes inflamed and necrosis occurs. Caused by bowel iscaemia and bacterial infection. Seen within the first few weeks of life. Early signs include feed intolerance and vomiting and bilious aspirates. Abdo becomes distended and stool can be blood stained. Can induce shock, and can lead to bowel perforation. Stop feed and give broad spectrum antibiotics, start Parenteral nutrition. Mechanical ventilation and circulatory support may be needed.
42
Normal ABG ranges?
pH: 7.35-7.45 pCO2: 4.7-6.0 pO2: 11-13 Bicarb: 22-26 BE: -2 to +2
43
Difference in type 1 and 2 resp failure?
Type 1: low O2 with no Co2 diff - V/Q mismatch: - Normal perfusion and reduced ventilation e.g.pulmonary oedema - Normal ventilation and reduced perfusion e.g. Pulmonary embolus Type 2: both O2 and CO2 are affected O2 is low, CO2 is high. - alveolar hypoventilation
44
Causes of Hyponatraemia?
Fluid status: - Low volume/Normal volume/High Volume Low volume: - D&V Normal volume, conc. Urine: - SIADH Normal volume, dilute urine: - adrenal insufficiency, Hypothyroidism, too much water High volume: - HF - Liver failure - Kidney failure
45
Causes of loose stools in a child?
IBD IBS (less likely) Long term infection Diet - too much fibre Addison's
46
Symptoms to ask if loose stools is presenting complaint?
Blood/mucus in stool Consistency Colour of poo Weight loss Abdo pain Intolerance to anything Coeliac? Difficult to flush away Smelly
47
What does fetal calpotectin test for?
Sensitive for IBD, not specific though
48
Types of spina bifida?
Occulta - most common, vertebrae don't properly form, doesn't usually cause issues Meningocele - meninges push out, will need surgery, outcomes aren't too bad. Myelomeningocele - most severe, spinal canal is actually open.
49
What is Hirschprungs disease?
Congenital megacolon Part/all of the large intestine has no nerve supply, causes blockage. Treated surgically by removing that bit of bowel.
50
What is congenital adrenal hyperplasia?
Collection of autosomal recessive genetic conditions that interfere with the production of steroids from cholesterol by the adrenal glands. Either results in excess or insufficient sex hormones.
51
Differential diagnoses for widening of the mediastinum?
Lymphoma Thymus Vascular anomalies
52
What is HSP (Henoch-Schonleine-purpura), what are it's clinical features?
IgA vasculitis Affects skin, mucous membranes of 3-13yr olds (ish), more common in boys, usually after respiratory infection. Classic symptomatic triad of 1. Purpura, skin rash 2. Arthritis 3. Abdo pain Can also get periarticular oedema and glomerulonephritis Clinical diagnosis based on combination of symptoms.
53
Hep B problem in children?
In children it can lead to chronic hep b infection (90% of children with hep b)
54
What is the retic count, in neonates?
The reticulocyte ("retic") count is the percent of RBCs that contain nucleic acid (also called "reticulated" cells), The retic count is used to assess whether the marrow is producing more RBCs than in the normal steady state. High in haemolytic disease Low in congenital anaemia
55
What is magnesium sulphate used for?
Bronchodilator in children Prevent eclampsia in pregnancy
56
What is a pyeloplasty?
Operation to remove a blockage from one of the ureters. Stent is put in and later removed.
57
Causes of hyponatraemia in neonates?
Administration of hypotonic fluids during the delivery. Renal failure Infection Gi fluid loss Adrenal insufficiency
58
Anatomical terms for a clear fluid filled area superficially on skin?
Vesicle - smallest <5mm Bulla - >5mm
59
Anatomical terms for a change in surface colour without elevation or depression?
Small (<5mm) - macula Larger (>5mm) - patch
60
Different anatomical terms for raised solid elevation of skin with no visible fluid?
Papule - <5mm Plaque - larger confluence of papules >1cm Nodule - similar to a papule (<5mm) but deeper in the dermis. Tumour - >5mm nodule
61
What is purpura? What is it called if it's smaller?
Non-blanching superficial, caused by broken blood vessels measuring 3-10mm Petechiae is the same but smaller than 3mm.
62
Causes of jaundice >24hrs?
Physiological jaundice - normal jaundice, caused by liver not working as well, and high levels of fetal haemoglobin breakdown. Breast milk jaundice - unconjugated bilirubin, normal Dehydration - in some infants IV fluids are needed. Caused by lack of bilirubin excretion? Infection BILIARY ATRESIA
63
Jaundice metabolism?
Haemoglobin breakdown to form unconjugated bilirubin. Unconjugated bilirubin is conjugated in the liver All of this conjugated bilirubin is excreted into the bowel. In the bowel bacteria form urobilinogen (some of this circulates back to the liver and some to the kidneys (where it is excreted in the urine) Urobilinogen in the bowel is further processed to form stercobilinogen - darker colour in the stool. Light stools - hepatic blockage (no stercobilinogen in stool) dark urine/stool - extra urobilinogen in urine - haemolysis
64
What is neonatal erythema toxicum?
Normal baby rash - will pass, central yellow plaque with a halo of erythema
65
What does the TORCH screen include
T- Toxoplasmosis O- Other - syphilis R- Rubella C- CMV H- Herpes
66
Measles rash and signs?
Koplik spots - appear as white spots in teh mouth before the rash does. Rash consists of confluenced macula (and sometimes papular) widespread - starts on the head/nech then spreads to rest of body.
67
Complications from chickenpox?
Ataxia from cerebellitis Encephalitis Rheumatitis GI involvement
68
What is impetigo? what does it look like?
Superficial skin bacterial infection caused most commonly by Staph A (but also strep & MRSA) Crusty yellow lesions of face arm and legs - may be painful/itchy
69
Common causative organisms of abscesses?
Staph and Strep
70
What is ichthyosis?
Family of rare genetic skin disorders, dry thickened scaly skin. Linked to metabolic syndromes such as T2DM and PCOS - have to screen.
71
What is aminophylline?
Methylxanthine, give via infusion - bronchodilator
72
What is clenil?
Beclomethasone
73
Principles of the UN statement on the rights of the child?
Right to survival Right to health and healthcare Right to develop to the fullest Right to full participation in family, social and cultural life Right to freedom from violence In the UK the 1989 and 2004 children act legislates for these rights
74
Definition of abuse and neglect?
Maltreatment of a child by inflicting harm or failing to act to prevent harm... In a family/institutional/community setting... By those known/not known to them... by an adult or child
75
Categories of abuse?
Physical abuse Sexual abuse Neglect Emotional abuse
76
Definition of physical abuse?
Any physical harm to a child, including when a parent or carer fabricates the symptoms of illness, or causes illness
77
Definition of sexual abuse?
Forcing or enticing a child to participate in sexual activities, whether they are aware of what is happening or not.
78
Definition of neglect?
Persistent failure to meet a childs physical or emotional needs, likely to result in the serious impairment of the childs health or development
79
Definition of emotional abuse?
Persistent emotional maltreatment of a child, such that it impairs the childs emotional development. Domestic violence for example.
80
How many 18-24 year olds report some kind of abuse?
11%
81
Toxic trio that potentially increase the risk of abuse
Domestic abuse Alcohol/drug misuse Mental illness
82
What should you do if you are concerned about a child?
Record full details of presenting incident, your concerns and observations Listen to child and put them first Seek advice from a senior Consider who else might be involved, siblings?
83
What type of immunity do vaccines produce?
Active immunity
84
What can vaccines be made up of?
Inactivated or attenuated live organism Secreted products Components of cell walls
85
What is the childhood immunisation schedule?
2 months: - DTaP - IPV - Hib - PCV - Men B - Rotavirus 3 months - DTaP - IPV - Hib - Rotavirus 4 months - DTaP - IPV - Hib - PCV - Men B 12 months - Hib - Men C - MMR - PCV - Men B 2-7 years - Influenza 3.5 - 4 years - DTap - IPV - MMR Girls 12/13 - HPV 14 Years - TdIPV - MenACWY
86
Aims of herd immunity?
- Reduce sources of infection - Those who cannot be immunised will still benefit - May eliminate disease
87
Contraindications for vaccinating a child?
For inactivated vaccines true anaphylaxis is the only contraindication Severe allergic reactions, stable neurological conditions, local or general reactions are all not contraindications Live vaccines - High dose steroids - Immunosuppressive treatment - hypogammaglobulinaemia - Pregnant - Had a live vaccine in previous 3 weeks
88
Which vaccines are inactivated
Pertussis Polio Tetanus Diptheria Pneumococcus Primary response - IgM then IgG There's a primary course then a booster
89
Which vaccines are live?
MMR Varicella Influenza Yellow fever BCG May see mild form of disease
90
Four main things in a 6 week health check?
Physical exam Review of development Give health promotion advice Opportunity for parent to express concern
91
What are you checking for in the physical exam in the 6 week baby check?
Congenital heart disease Developmental dysplasia of the hip Congenital cataracts Undescended testes
92
How would you check for congenital heart disease in teh 6 week check?
Look for cyanosis, ventricular heave, respiratory distress, and tachypnoea; a respiratory rate persistently over 55 is suspicious. Feel for apex beat and assess whether displaced. Listen for murmurs. Innocent murmurs are common and are typified by low intensity, localised to a small area of precordium and in the absence of other symptoms or signs. All murmurs should be referred to a specialist for assessment.
93
How would you check for developmental dysplasia of the hip (in the 6 week check)?
leg-leg length discrepancy Asymmetry of leg creases Barlow and Ortolani tests - Barlow: press both thighs posteriorly looking to see if femoral head dislocates - Ortolani: looking to see if femoral head pops back in If abnormality should have USS
94
Eye exam in the 6 week check?
Examine external eyes (one eye bigger may indicate glaucoma) Check for normal red reflex Check for FH, ask if parents have any worries Any abnormalities should be treated as urgent referral, abnormal red-reflex should be same day
95
How would you assess the development of the baby in the 6 week check?
Review feeding and weight gain Check growth chart Review vision and hearing Most will be spontaneously smiling, and making a variety of sounds e.g. coos
96
What health promotion discussions should you have in the 6 week check?
Immunisations Breast feeding, check tongue tie SIDS - No smoking - Sleep on back (but have tummy time) - Don't sleep on same bed/sofa together - Avoid overheating - Breast feed Could ask about maternal depression
97
Common physical signs of abuse?
Abnormal bruising Cuts and scratches Bite marks Unconscious or fitting (shaken baby) Subtle signs such as sky away from touch, not want to get changed for PE, afraid to go home.
98
What would constitute abnormal bruising?
- <1 year (as not walking) - Disproportionate to reason given - Multiple sites or clusters - Look like a hand, stick, tooth, belt - Non bony part of the body or face e.g. eyes, ears, cheek, back, abdomen - Ankles or hands - On neck - Delay in presentation
99
Childs behaviour that may suggest sexual abuse?
Anxious about going to a particular place or seeing a particular person. Sudden mood swings, or aggressiveness Displays over-sexualised behaviour or inappropriate knowledge for their age
100
Who is the usual suspect for sexual abuse?
Usually the abuser is a family member or someone known to the child, such as a family friend. For teenagers it is commonly a boyfriend or girlfriend. Child sex abusers can come from any professional, racial or religious background, and can be male or female. Older children may abuse younger children.
101
4 areas of early development
Gross motor Vision and fine motor Hearing, speech and language Social, emotional and behavioural
102
When do you stop correcting for prematurity?
At 2 years post-natal
103
Rough guide to gross motor development 12 months up until 3 years?
6-8 weeks - Moves head side to side when prone - developing head control 6-8 months - sits briefly - rolls (both ways) - starts to crawl 12 months - walks unsteadily 18 months - squats to pick things up off the floor - Runs - Walks well 2 years - Kicks ball - Climbs stairs 2 feet at a time 2.5 years - Running well 3 - Jumps with both feet - Stairs 1 foot at a time 4-5 - Stands on one foot - Throws and catches
104
Fine motor/vision rough guide from 14 months to 4 years?
6-8 weeks - grasp reflex - hands lightly closed 6-8 months - Palmar grasp - Object from hand to hand - Reaches for toys 12 months - Mature grasp - Gives objects away 18 months - Linear scribble - Tower of three 2 years - Tower of six - Circular scribble 2.5 - Tower of eight 3 years - Circle - Bridge 4 years - Square - Steps after demonstration - Man with head, legs and trunk 5 years - Triangle - Person with 6 body parts - fork and spoon
105
Rough guide to communication and hearing 12 months to 3.5 years?
6-8 weeks - Coos and gurgles - Watches mums face - Startled by loud noises - Cries when hungry or uncomfortable 6-8 months - Turns to mum - laughs aloud 12 months - can say single words, probably two or three in vocab 18 months - Can point - 6-10 words, knows two areas of the body 20-24 months - Simple phrases using two or more words 2.5 - 3 years - Talks all the time in 3-4 word sentences 4-5 years - Speech grammatically correct - Nursery rhymes - Name and age
106
Rough guide to social, emotional and behaviour development?
6-8 weeks - Smiles - follows people with eyes 6-8 months - Takes everything to mouth - Plays with rattle - Looks at self in mirror 12 months - Holds spoon, puts to mouth - waves bye-bye - Demonstrates affection 18 months - Symbolic play beginning - Uses a spoon 24 months - Dry by day - Pull some clothes off - Toddler tantrums - No awareness of danger 3 years - Turn taking - Vivid interactive play with others - Names friend 4-5 years - Dresses and undresses - Complex games - Comforts others - independent toileting
107
What are learning disabilities and learning difficulties?
Learning disabilities is the general condition of arrested development, resulting in the impairment of skills manifest during the developmental period. Learning difficulties applies to a specific area of difficulty e.g. dyslexia or dyscalculia
108
Different grades of learning disabilities?
Mild (IQ 50-69) Moderate (35 - 49) Severe (20-34) Profound (<20)
109
Learning difficulty causes?
Prenatal - Genetic abnormalities - Intrauterine abnormalities - Intrauterine insults Perinatal - Prematurity - Severe asphyxia - meningitis Postnatal - Infection - Trauma - Lead poisoning - Malnutrition - Neglect
110
What is neurodisability?
Group of congital or acquired disorders including impairment to the brain or neuromuscular system, affecting: Movement, Cognition, Hearing or vision, communication and emotion/behaviour
111
General structure to an assessment of development?
History - Parents concerns - Is there a barrier to learning? - Is there a useful diagnosis to be made? Reports - Nursery - School - physio - SALT Observation and informal development - may be different to testing situation Formal evaluation - Schedule of growing skills - Griffiths (toys) - Bayley infant development scales - Hammersmith infant neurological examination
112
Clinical signs in a neuro-developmental assessment?
Patterns of growth Dysmorphic features Neurocutaneous stigmata CNS abnormalities - Tone - Wasting - reflexes CVS abnormalities Visual abnormalities Hearing abnormalities - Ask parents - Check tests were done as newborn Patterns of mobility - dexterity - hand dominance
113
Causes of abnormal development (broadly)?
Neglect of physical and psychological needs Ill health Neurodevelopmental disorder
114
Causes of abnormal development (broadly)?
Neglect of physical and psychological needs Ill health Neurodevelopmental disorder
115
When does global developmental delay normally present?
Within the first two years
116
Causes of abnormal motor development?
Central motor - Cerebal palsy Congenital myopathy/primary muscle disease Spina bifida Global developmental delay - Syndromes - Unidentified cause
117
If a child showed hand dominance or asymmetry of motor skills within the first year is this okay?
No, hand dominance isn't present until 1-2 years, and this may suggest a underlying hemiplegia
118
When is the limit age of walking? When might this be less worrying?
>18 months If the child shows the variants of bottom shuffling or commando crawling this may be normal.
119
What is cerebal palsy?
An abnormality of movement or posture caused by disturbances in the developing fetal or infant brain. Used up until about 2 years old then would call it acquired brain injury Often accompanied by disturbances of - cognition - communication - perception - sensation - behaviour - seizure disorder
120
What are the causes of cerebral palsy
80% antenatal - Vascular occlusion - Cortical migration disorders - Structural maldevelopment Genetic syndromes Infection 10% due to hypoxic injuries at delivery About 10% postnatal - CNS infection - Head trauma - Hypoglycaemia - Hyperbilirubinaemia - Hydrocephalus
121
Early features of cerebral palsy?
Abnormal limb/trunk posture and tone Feeding difficulties Abnormal gait Asymmetric hand function
122
Types of cerebral palsy?
Spastic - UMN - Increased limb tone Dyskinetic - involuntary, uncontrolled movements Ataxic - hypotonia and poor balance
123
Causes of speech and language abnormalities?
Hearing loss GDD Anatomical problem e.g. cleft palate Environmental deprivation Normal variant
124
What type of neuro-developmental deal would downs cause?
Global developmental delay
125
When does autism present?
2-4 years
126
Management of autism spectrum disorder?
Applied behavioural analysis is the only intervention really shown to work
127
What is development co-ordination disorder or dyspraxia?
Disorder of motor planning and/or execution, without any findings on a neurological examination, a disorder if higher cognitive functions it is the inability to execute a planned series of actions. Difficulties can present as: - Handwriting - Dressing (buttons etc) - Cutting up food - poorly established laterality - copying and drawing - Messy eating
128
What is dyslexia?
Disorder of reading skills disproportionate to the child's IQ, need to include vision and hearing assessment.
129
Causes of in-toeing in children?
Metatarsus varus - passively correctable - no treatment required Medial tibial torsion - toddlers - self corrects in 5 years Persistent anteversion of the femoral neck - usually self corrects by 8 - if persistent can do femoral osteotomy
130
Types of muscular dystrophy?
Duchenne Becker Congenital
131
Inheritance pattern of duchenne muscular dystrophy?
X-linked recessive
132
Brief summary of the pathophysiology of duchenne's?
Abnormality in coding for the dystrophin protein. Leads to malformation of muscle fibres.
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What blood result is high in duchennes muscular dystrophy?
CPK creatine phosphokinase
134
When are duchenne's patients normally first noticed?
5.5 years
135
Presentation of duchenne's?
Waddling gait +/- language delay Pseudohypertrophy of calves Clumsier and slower than peers
136
Progression of duchennes?
Present 5-6 years In a wheelchair by 10-14 Respiratory failure and associated cardiomyopathy Life expectancy is late 20s
137
Management of duchenne's muscular dystrophy?
Physio - Appropriate exercises to maintain muscle power and delay the onset of scolios Contractures, particularly at the ankles should be prevented by passive stretching and wearing of splints Later CPAP may be required, or NIPPV
138
Brief differences in becker muscular dystrophy?
Some functional dystrophin is still produced, similar features to duchenne's, but will progress slower (age of onset is 11 y/o), life expectancy is 40 or normal
139
Features of congenital muscular dystrophy?
Recessively inherited heterogenous group of disorders. Normally presents at birth with weakness, hypotonia or contractures
140
How common is down syndrome in the UK?
1 in 1000 births
141
Most common form of inheritance for down syndrome?
Non-dysjunction
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What is non-dysjunction?
Error at meiosis (sex cell production producing haploid cells. Chromosome 21 from the parent cell does not divide producing one gamete with two 21's and one with none.
143
How can down syndrome otherwise be inherited (not non-dysjunction)?
Translocation - 21 is joined onto another chromosome (normally 14) - Can produce carriers Mosaicism - Some cells trisomy some not
144
How is down syndrome screened for antenatally?
Combined test offered at 10-14 weeks - PAPP-A - Nuchal translucency scan - β-hCG
145
Facial features of down syndrome postnatally? (10)
Round face with flat nasal bridge Upslanted palpebral fissures Epicanthic folds Brushfield spots in iris Small mouth and protruding tongue Small, low-set ears Flat occiput and third fontanelle. Short neck
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Other (non-facial) symptoms of down syndrome?
Single palmar crease incurved 5th finger wide gap between Big and 2nd toe
147
Features of turners syndrome?
Short stature Webbed neck Low set ears Broad chest
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Features of edwards syndrome?
Microcephaly Prominent occiput Narrow palpebral fissures Cleft lip/palate Low set ears
149
Features of fetal alcohol syndrome?
Smooth philtrum Thin upper lip Small palpebral fissures Single palmar crease
150
Complications associated with downs?
Cardiovascular complications VSD. ASD PDA Aortic Coarctation MVP Tetralogy of Fallot Duodenal atresia Hirschprungs disease
151
usual age for presentation of diabetes in children?
above 10, between 10-14 yrs normally
152
Adverse health outcomes for children with diabetes?
Reduced life expectancy - 20 years in T1DM - 10 years in T2DM Increased risk of stroke and MI Renal failure Blindness Stillbirth/miscarriage/congenital malformations
153
Findings on urine dipstick in childhood type 1 diabetes?
Glycosuria, ketonuria (can be normal if not glycosuria, in diabetes may be indicative of DKA.
154
Treatment for T1DM in kids?
Fast acting insulin - novorapid Slow acting insulin - Glargine Mixed Basal bolus regime - Long acting at night - Short acting Mixed regime - two injections 10 hours apart
155
Hypoglycaemia blood glucose in a patient on insulin?
<4mmol/L (<2.7mmol/L in others)
156
What would you tell a child to look out for in hypoglycaemia?
Sweating Fatigue Feeling dizzy
157
Is it common to be in DKA when presenting for T1DM as a kid?
Yes, 2/3 kids are at presentation.
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DKA presentation?
Polyuria Polydipsia Breathless Tired Weight loss N&V Headache Ketotic breath Shock Drowsy Abdo pain Constipation
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What type of acid base disturbance would DKA cause?
Metabolic acidosis, (N&V, Dehydration?)
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Three stages of paediatric growth?
Infant (0-2) - rapid growth - nutrition deoendent Childhood (2-puberty) - Steady growth - Hormone dependent Pubertal growth - Rapid linear growth - Depends on pubertal hormones and GH - Start of puberty in girls - End of puberty in boys
161
When should you refer a child for poor growth?
Short for parents height (mid-parental height) Height velocity is below that for age Crossed >2 centiles up or down Height <0.4th centile
162
Average age of puberty in girls and boys?
10.5 - girls 14 - boys
163
What age is precocious puberty in boys and girls?
<8 in girls <9 in boys
164
What age is pubertal delay in boys and girls?
>13 in girls >15 in boys
165
What is arrested puberty for boys and girls?
Time to complete puberty is too long Girls should complete in 4 years Boys should complete in 6 years
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Type of conditions that can cause abnormal growth and development?
Chromosomal abnormalities Malnutrition - poor intake or malabsorption Chronic ill health Iatrogenic Psychological & emotional factors Skeletal abnormalities Syndromes Hormones
167
How would coeliac disease present in terms of growth and development?
Abdo distention Discrepancy between parental and child's height Drop in weight following weaning
168
If a kid is fat and tall what's most likely the diagnosis?
Exogenous obesity
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If a kid is short and fat what's the most likely diagnosis?
Endocrine disorder
170
How might hypothyroidism present in childhood?
Short stature - thyroxine is important for growth Might be cold Dryness of the skin and hair, coarsening of the facial features, constipation and a slow pulse rate all occur in children but tend to be relatively late features.
171
In terms of growth and development how might turners present?
Pubertal delay and short stature.
172
Ddx if a girl of 5 y/o started developing pubic hair?
Increased adrenal hormone secretion Precocious puberty Premature Adrenarche (normal variant)
173
Chromosomal abnormalities associated with abnormal growth?
Downs - short Klinefelters - XXY, boys may grow more quickly Turners - short/no puberty
174
Nutritional disorders causing abnormal growth?
GORD - feeding aversion Coeliac - short IBD Pancreatic insufficiency - CF
175
Most common skeletal abnormality causing poor growth?
Achondroplasia
176
Genetic syndromes associated with short stature?
Noonans - cardiac and developmental delay Williams - elfin appearance, warm and bubbly, short stature and cardiac defects Russel-silver syndrome - poor growth
177
Hormonal problems resulting in abnormal growth?
Hypothyroidism - short and fat GH deficiency - short and slightly chubby Hypopituitarism - short
178
What can affect GH?
Stress Poor sleep Malnutrition SOL in pituitary
179
What investigation would you want to do in precocious puberty?
Image pituitaries
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If a kid is gillick competent can they refuse consent given on behalf of them?
No - can only give consent
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What are you checking for in a newborn infant assessment?
Birth weight - centile & age plotted Posture & movements Head circumference Fontanelles - if tense and no crying need to exclude hydrocephalus Face - genetic abnormalities Pale - check anaemia Jaundice - <24 hrs is abnormal (haemolysis) Red reflex - cataracts - retinoblastoma Palate - cleft RR, auscultate heart Abdo masses Femoral pulse - coarctation of the aorta Genitalia and anus - check wee and poo Muscle tone Back & spine Hips - the two tests (barlow and ortolani)
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What is hypoxic ischaemic encephalopathy?
ischaemic injury occurring perinatally at the time of labour or delivery resulting in brain damage.
183
Common causes of hypoxic ischaemic ancephalopathy/
Excessive/prolonged contractions (hyperstimulation?) Cord compression (shoulder dystocia & prolapse) Hypo/hypertension IUGR/anaemia Failure to breathe
184
What causes RDS in neonates?
Surfactant deficiency
185
Management of neonatal jaundice?
Phototherapy - blue-green light converts unconjugated bilirubin into a harmless water soluble pigment Exchange transfusion - at higher levels of bilirubin - blood from art line or umbilical vein is removed, and replaced with donor blood.
186
Signs of respiratory distress syndrome in the newborn?
Same as normal respiratory distress: - Head bobbing - Nasal flaring - Grunting - Recession (4 types) - Tachopnoea (>60 RR) - Cyanosis
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Presentation of IVH in the newborn?
Intraventricular haemorrhage Symptoms: - Diminished/absent Moro reflex. - Poor muscle tone. - Sleepiness. - Lethargy. - Apnoea. Signs: - The fontanelle may be tense and bulging with severe IVH. - Neurological depression may progress to coma. In mild forms there may be no clinical signs, or there may be alternating symptomatic and asymptomatic periods.
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Management of IVH in the newborn
Intraventricular haemorrhage Management is initially mainly supportive and may include the correction of anaemia, acidosis and hypotension. Ventilatory support may also be required for some who deteriorate acutely. Fluid/volume replacement: - Packed red blood cells or fresh frozen plasma for anaemia and shock. - Sodium bicarbonate infusion (carefully) for metabolic acidosis.
189
What is the APGAR score how do you calculate it?
(named after it's creator) A - appearance P - Pulse (>100 N) G - Grimace (movements) A - Activity (Strong/weak) R - Respiration (cry/no cry) 0 is nothing 1 is something 2 is normal
190
What is transient tachypnoea of the newborn?
A self-limiting period of respiratory distress most often seen in term babies following pre-labour Caesarean Section. It is thought to be due to delayed absorption of lung fluid and may require oxygen therapy.
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What is meconium aspiration?
It is not uncommon for infants to pass meconium before delivery. In rare instances the infant may aspirate meconium stained liquor, this occurs when a hypoxic infant begins to gasp in utero. Pulmonary vasodilatation is delayed and results in Persistent Pulmonary Hypertension which can make an infant very unwell.
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Why do we give vit k to babies?
To prevent haemorrhagic disease of the newborn - deficiency in factors II, VII, IX, X1
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What is cephalohaematoma in the newborn?
Subperiosteal haematoma (can't cross suture line) resolves over weeks, may exacerbate jaundice
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What is a caput in the newborn?
Oedema and bruising usually associated with a ventousse delivery, resolves in a few days and can cross suture lines
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Benefits of breast feeding?
Nutrition, protection from disease, development, allergy, autoimmune, reduced obesity and long term health.
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General advice regarding the continuation of breast feeding and the administration of bottle fed milk?
Breast feeding is the gold standard; the World Health Organisation recommends that infants should be exclusively breast fed until 6 months of age Solids should not be introduced before 4 months Bottles for formula milk must be sterilised and milk made up according to instructions with boiled water
197
Causes of prolonged jaundice
Most commonly breast milk jaundice Although must rule out biliary atresia - pale stools - dark urine
198
Infants at risk of GBS infection?
Premature If the mother is colonised with Group B strep or has previously had a child with Group B strep infection There is evidence of maternal infection (pyrexia or elevated CRP) There is prolonged rupture of membranes Evidence of fetal distress
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Four infections mothers are screened for?
hepatitis B, HIV, syphilis and susceptibility to rubella
200
When is the blood spot screening performed, what is it testing for?
PKU Congenital Hypothyroidism Sickle cell disease MCADD CF Isovaleric acidaemia Maple syrup urine disease Homocystinuria Glutaric Aciduria type 1(GA1)
201
What type of bilirubin crosses the BBB?
Unconjugated bilirubin, this occurs in kernicterus
202
What does the ductus arteriosus do antenatally? What is the problem in PDA?
Shunts blood from the pulmonary artery to the aorta. In PDA this means there is less volume of blood in the aorta
203
Presentation of PDA in a premature infant?
Classical signs are usually absent May be systolic murmur in left sternal edge Bounding peripheral pulses Hyperactive precordium (can see it moving) Tachycardia with or without gallop rhythm
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Investigations for suspected PDA?
Echocardiogram
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Management of PDA in the preterm infant?
Possible NSAIDS ?indomethacin Conservative Surgical closure - may be associated with increased morbidity
206
Presentation of NEC in the prem baby?
Abdominal distension with increasing gastric aspirates. Altered stool pattern. Bloody mucoid stool and bilious vomiting. Decreased bowel sounds with erythema of the abdomen. Palpable abdominal mass or ascites. Associated features are bradycardia, lethargy, shock, apnoea, respiratory distress, temperature instability.
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Investigations for NEC?
Bloods - FBC and blood gas, serial CRPs Abdo XRAY confirms findings/not
208
Management of NEC?
Nil by mouth TPN, IV fluids and Abx surgery inc. bowel follow through can be performed
209
Most common cause of ambiguous genitalia in newborns?
Congenital adrenal hyperplasia
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Diagnosis of hirschsprungs disease?
Rectal biopsy
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Presentation of hirschsprungs disease?
Delayed passage of meconium (> 2 days after birth), abdominal distension and bilious vomiting. May have distended abdomen, and bowel movements not working otherwise - visible waves of peristalsis.
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Causative organism in scarlet fever?
Group A haemolytic streptococci
213
How do left to right shunts commonly present?
Breathless
214
How to right to left shunts commonly present?
Blue
215
inheritance pattern of haemophilia?
X-linked recessive
216
Causes of left to right shunts?
VSD PDA ASD
217
Causes of right to left shunts?
Tetralogy of fallot Transposition of the great arteries
218
Innocent murmurs?
The S's aSymptomatic patient Soft blowing murmur Systolic only left Sternal edge
219
ASD presentation?
None (common) Recurrent chest infections / wheeze Arrhythmias Ejection systolic murmur on upper left sternal edge
220
Management of ASD?
Correction by 3-5 years of age in ASDs that are significant enough to cause right ventricle dilatation
221
Presentation of a VSD?
Small: - asymptomatic - Loud pansystolic murmur left lower sternal edge CXR, ECG will be normal Echo will show defect Large: - HF, breathlessness, recurrent chest infections Tachypnoea, tachycardia, hepatomegaly Active precordium Pansystolic murmur CXR - cardiomegaly - Enlarged pulmonary arteries - Pulmonary oedema ECG - Biventricular hypertrophy Echo - shows defect
222
VSD management?
Small may manage conservatively First line is increased calorific density of feeding – orally if they can tolerate it, and if they can’t manage oral feeds can supplement with NG feeds. Don’t give fluids – more volume, more work – exacerbate the problem Manage HF: - Diuretics - Captopril ACEI Digoxin (but not really)
223
What is a PDA (in a term infant)?
When the PDA has not closed 1 month after EDD
224
PDA clinical features?
Continuous murmur beneath the left clavicle Wider pulse pressure - collapsing or bounding pulse Large PDA may cause HF & pulmonary hypertension CXR and ECG normally normal
225
Management of PDA >1month
Close it with coil or occlusion device
226
Four features of tetralogy of fallot?
Large VSD Overriding aorta Subpulmonary stenosis RV Hypertrophy
227
Presentation of tetralogy of fallot?
severe cyanosis hypercyanotic spells squatting on exercise Clubbing Loud ejection systolic
228
Management of tetralogy of fallot?
Neonates - prostin to keep ductus arteriosus open Surgically - shunt - RVOT stent (more common now) if less severe can perfom surgery 3-6 months in attack: - On shoulder with calming - Otherwise give O2 and morphine +/- intravenous propranolol.
229
What are AVSDs most commonly associated with?
Trisomy 21
230
Common genetic abnormality associated with fallots?
22q 11 deletion
231
What is coarctation of the aorta?
Narrowing of the aortic arch
232
Clinical findings in coarctation?
Hypertension in upper vasculature - arm
233
Treatment of coarctation?
Prostin to maintain ductus arteriosus and supply lower half of body Then surgery in first few weeks to correct If narrowing is less, may present late - Notching on CXR - Absent femorals - HTN upper body
234
Management in transposition of arteries/
Prostin to keep ductus arteriosus open Sugical operation called balloon atrial septostomy - shunt in atrium Later (2 weeks) do arterial switch proceadure
235
What Is HLH in children how do they present and how do you manange?
Hypoplastic left heart - very rare Pesent dueing antenatal screening usually or at birth will be very sick with no peripheral pulses Need to do norwood proceadure
236
What is aortic stenosis in newborns?
Aortic valve leaflets are fused together can present with other abnormalities such as coarctation of the aorta
237
How does aortic stenosis in newborns present?
Asymptomatic murmur, ejection systolic (upper right sternal edge) Carotid thrill Ejection click
238
Management of aortic valve stenosis?
Monitor early to assess when to intervene then balloon valvotomy, less complicated when older. If problems such as intolerance to exercise may have to do younger, will probably eventually need to replace aortic valve
239
Interval symptoms in asthma?
Night time cough Wheeze on exertion
240
Diagnosis of asthma in children, 3 cardinal symptoms?
Breathlessness Wheeze Cough
241
What might asthma show on spirometry?
15% improvement on reversibility
242
Treatment of asthma?
Preventers - ICS - LABA - Leukotriene receptor antagonist Relievers - SABA - Ipratropium bromide Start with SABA, then ICS then LABA/Leukotriene then more steroid
243
Genetic abnormality in CF and inheritance pattern?
ABnormality in the CFTR chloride channel on chromosome 7 causing thick mucus secretions 1 in 20 people are carriers Autosomal recessive
244
How does CF present?
20% at birth with bowel obstruction (meconium ileus) Older - cough - recurrent infection - fatty light stool (pancreatic insufficiency) - failure to thrive
245
CF management?
Physiotherapy (at least twice a day) High calorie diet Pancreatic enzyme supplements Psychological input
246
Bronchiolitis management?
Supportive, O2 therapy, High flow CPAP Ventilation NG, IV fluids if needed
247
Bronchiolitis presentation?
Couple of days of coryzal illness (longer than VIW) Irritability, feeding difficulty Cough, wheeze (expiratory) , crackles
248
Croup (upper airway obstruction) presentation?
Stridor Barking cough Hoarse voice <38.5 don't look toxic
249
Persistent upper airway obstruction (with stridor) most likely caused by what?
Laryngomalacia
250
What does laryngomalacia look like?
Omega shaped deformity of the epiglottis
251
most common cause of croup?
Parainfluenza virus
252
Croup management?
Don't look at throat Keep child calm Oxygen and hydration Dexamethasone steroid orally Nebulised budesonide Nebulised adrenaline Intubate
253
Croup management?
Don't look at throat Keep child calm Oxygen and hydration Dexamethasone steroid orally Nebulised budesonide Nebulised adrenaline Intubate
254
How can you (maybe) differentiate between viral and bacterial pneumonia?
Viral: - more common in infants - fever <38.5 Bacterial - Fever >38.5 - Any age - Resp rate >50
255
Presentation of pneumonia in children?
Fever Tachopneoa Cough O/E: - Dullness - Reduced air entry - Bronchial breathing
256
Investigations and management of kids with pneumonia?
Investigations: - sputum analysis - bloods - blood culture - CXR - URINE PNEUMOCOCCAL ANTIGEN Tx - Oxygen - Physio if collapse (of lung) - Abx If empyema then chest drain and urokinase enzyme
257
What is whooping cough, how does it present, how do you treat?
Highly contagious infection caused by pertussis Week of coryzal symptoms then spasmodic cough with inspiratory whoop
258
Viral induced wheeze in children treatment?
Bronchodilators and steroids as well as supportive care.
259
Recurrent cough Ddx in children?
Series of respiratory tract infections Asthma CF Reflux Foreign body TB Habit
260
How many Obstructive sleep apnoea present in children?
Loud snoring Witnessed apnoeas (stopped breathing) Restless and disturbed sleep Can cause daytime sleepiness leading to learning and behavioural problems
261
What is obstructive sleep apnoea normally caused by?
Adenotonsillar hypertrophy
262
Key investigation for sleep apnoea on children?
overnight pulse oximetry, however normal oximetry doesn't exclude the condition and may have to do polysomnography
263
General Ddx for vomiting?
Reflux Feeding issues Infection - Gastro/elsewhere Intolerances Obstruction Metabolism CAH Renal failure Appendicitis Raised ICP Coeliac disease Migraine Anorexia Pregnancy Testicular torsion
264
When does reflux (GORD) normally resolve in children?
By 12 months, normally by 6 months.
265
Complications of reflux in children?
Failure to thrive Oesophagitis - haemetemesis - Iron deficiency anaemia Pulmonary aspiration Apparent life-threatening events
266
What is colic in kids?
Colic is a frequent crying in a baby who appears to be otherwise healthy and well fed. It occurs in 1 in 5 babies and affects male and female infants equally Usually begins within the first few weeks of life resolving by 4-6 months. Crying tends to be worst in the late afternoon or evening and usually lasts for several hours. During episodes the baby's face may become flushed, and they may clench their fists, draw their knees up to their tummy, or arch their back. Linked to reflux and migraines.
267
What causes reflux in babies?
Liquid diet Immature lower oesophageal sphincter Predominantly lying down Short distance from mouth to stomach
268
Reflux management?
Conservative (depends on severity) - Don't worry - Sit up - Thicken feed. Medical: - Ranitidine (5HT anatag) - PPI
269
How does cows milk protein allergy present?
Atopic eczema Gastro-Oesophageal Reflux Disease Chronic Gastro-intestinal symptoms, including chronic constipation
270
How would pyloric stenosis present?
Around 3 weeks Worsening vomits with feeds, progressing to projectile vomiting, non-bilious with undigested milk. Mallory-Weiss tear may lead to blood in vomit. Weight loss, dehydration, reduced urine output and even shock. Infants may become jaundiced; resolves with treatment. Hypochloraemic Metabolic Alkalosis Persistent vomiting leads to a loss of hydrogen and chloride ions, leading to a Hypochloraemic Metabolic Alkalosis. There is increased reabsorption of Bicarbonate in the kidneys whilst passive Na reabsorption is hindered by chloride loss; active reabsorption is done at the expense of Potassium ions which, along with vomiting losses, leads to Hypokalaemia (this may initially be concealed due to a potassium shift from extracellular fluids).
271
Pyloric stenosis management?
Initial management includes placing the child nil by mouth and correcting the dehydration and electrolyte imbalance. The child can then go to surgery for a Pyloromyotomy - a laparoscopic procedure where the thickened muscle is cut to allow widening of the stomach outlet. The infant is usually able to feed within 6 hours and makes a full recovery with no ill effects.
272
Test for cows milk protein allergy?
• Take it away for 3-4 weeks • AND • GIVE IT BACK!!!!!!!!!!!
273
Overflow constipation management?
Movicol first line with escalating regime Then add stimulant laxative Add lactulose if hard stools continue treatment for a few weeks after regular habit established and taper down slowly
274
What do you need to check with constipation?
Leg weakness/delay gross motor development (spinal abnormality) Constipation from birth Faltering growth
275
What investigations are available for coeliac disease, when would you perform them?
TTG and total IgA are first line serological tests. MUST be done whilst the patient is eating gluten. Definitive diagnosis is a biopsy - needs to be done whilst on gluten - shows subtotal villous atrophy
276
Features of crohns disease?
Diarrhoea Abdo pain Oral ulceration Rectal bleeding Anal tags, fissure and fistulae Weight loss Poor growth and delayed puberty
277
Features of ulcerative colitis?
RECTAL BLEEDING PASSAGE OF MUCUS DIARRHOEA URGENCY ABDOMINAL PAIN POOR GROWTH &DELAYED PUBERTY
278
Manifestations of IBD outside the bowel?
JOINTS: Arthritis can occur, leading to painful swollen joints e.g.knees hips ,ankles. SKIN : “Erythema Nodosum”- red painful lumps on the lower legs ,shins and occasionally the arms. EYES : “Iritis” - inflammation of the iris. “Episcleritis”- inflammation of the eyeball. LIVER : Inflammation and scarring of the liver and bile ducts.
279
Difference pathophysiologically between Crohns and UC?
UC: - Affects colon only - Diffuse progression from rectum upwards - Mucosal involvement - Histology – crypt abscesses / pseudopoyps Crohns - Can affect anywhere along the GI tract – pan enteric - Skip lesion - Transmural involvement - Histology – granulomas/ cobble stone appearances
280
Confirmation of IBD diagnosis?
Upper and lower colonoscopy with biopsy.
281
Big risk in UC?
Toxic megacolon
282
Toxic megacolon management?
1. IV fluids and NBM 2. IV antibiotics 3. IV steroids 4. Blood transfusion if necessary 5. Urgent surgical review (N.B. emergency colectomy has a mortality of 10%, but perforation has a mortality of 33%)
283
What is toxic megacolon?
Toxic megacolon is an acute form of colonic distension. It is characterized by a very dilated colon (megacolon), accompanied by abdominal distension (bloating), and sometimes fever, abdominal pain, or shock.
284
What is toddler's diarrhoea?
Toddler's diarrhoea typically occurs in the second year of life and is associated with undigested food such as peas and carrots in the stools. The child is well and growing normally. It is thought to relate to a rapid intestinal transit time. It resolves by the age of 4 years.
285
What is intussusception?
Describes the invagination of the proximal bowel into the distal segment - commonly ileum into caecum at the ileocaecal valve
286
When does intussusception usually occur?
3 months to 2 years
287
Complications in intussusception?
Venous obstruction causing engorgement, bleeding and bowel perforation
288
Presentation of intussusception?
Paroxysmal severe colicky pain and pallor May refuse feeds, vomit - may be bilious Redcurrent jelly stool Sausage shaped mass in abdomen
289
Management of intussusception?
IV fluid resus Rectal air insufflation - by a radiologist Surgically if the air fails
290
Abdominal migraine presentation?
Periodic pain, can be in the midline assoc. with vomiting and facial pallor Headache is normally present too, but not necessarily
291
Gastroenteritis pathogens?
Most common - rotavirus (up to 60%) Others: - adenovirus - norovirus - coronavirus - astrovirus Bacteria are less common, may have blood in the stool - Campylobacter jejuni - Shigella and salmonella - blood and pus - Cholera and e. coli - profuse rapidly dehydrating
292
groups of children at risk of dehydration with respect to gastroenteritis?
<6 months or born with low birth weight >6 diarrhoeal stools in prev. 24 hrs > 3 vomits in previous 24 hours Not tolerating fluids Malnourished
293
Signs of dehydration in children?
Decreased conscious level Sunken fontanelle Dry mucous membranes Sunken eyes Tckypnoea prolonged cap refill Tchycardia Pale skin Hypotension Weight loss Cold extremities Reduced tissue turgor
294
Management of gastroenteritis?
May get stool sample if in shock Hypernatraemic dehydration is hard to manage: - Oral rehydration solution IV fluids (if shock) - need to measure plasma sodium, and if hypernatraemic then slow infusion, over 48 hours. Antibiotics if there is sepsis
295
Gastroenteritis presentation?
D & V, may be evidence of infection such as contact with an infected person or travel abroad.
296
What amount of ORS do you give in management of dehydration?
50 ml/kg over 4 hours
297
How do you correct fluid deficit (in IV fluids) in kids - not maintenance?
100ml/kg if shocked 50ml/kg if not shocked 0.9% saline or normal saline with 5% dex
298
most common pathogens causing UTIs in children?
E coli - almost all Klebsiella Proteus species
299
Risk factors for UTIs?
Constipation - incomplete voiding, urinary stasis Voiding dysfuntion - Sit on heel in girls, pinch penis in boys, so that they don't have to go to the loo, may not empty properly urinary stasis again. Anatomic anomoly -PUJ (Pelviureteric junction obstruction) - others Neuropathic - spina bifida 1 year fairly common - not potty trained, need basic hygiene advice perhaps > 6 months more common in girls <6 month more common in boys
300
Presentation of UTI?
Preverbal (<3 months) - fever - vomiting - lethargy - irritability - poor feeding - tenderness > 3 months (if verbal ) - dysuria - frequency - abdo/loin tenderness - dysfunctional voiding
301
Management of UTI?
Really ill - pyelonephritis - Analgesic - fluids - IV abx Well child - Abx - ?analgesia
302
Types of enuresis
Primary nocturnal enuresis Daytime enuresis (normally with night) Secondary enuresis
303
What is daytime enuresis?
lack of bladder control during the day in a child over 3-55 years
304
What can cause daytime enuresis?
Lack of attention to bladder sensation, normal or developmental or psychogenic Detrusor instability Bladder neck weakness Neuropathic bladder UTI Constipation Ectopic ureter
305
Management (inc. investigations in daytime enuresis?
Examination to rule out neuropathic bladder Urine sample for microscopy to exclude UTI USS of bladder urodynamics Can use star reward charts, bladder training and pelvic floor exercises if neuropathic bladder or anomaly excluded
306
What is secondary enuresis?
Loss of previously achieved urinary continence
307
Causes of secondary enuresis?
Emotional upset (most common) UTI Polyuria from diabetes or CKD
308
management of secondary enuresis (inc. management)?
Test urine sample for infection, glycosuria and proteinuria USS of renal tract Assess urinary osmolality of early morning sample to assess urinary concentrating ability
309
Causes of proteinuria?
Orthostatic proteinuria - when standing up, will resolve Glomerular abnormalities Increased glomerular filtration pressure Reduced renal mass HTN Tubular proteinuria
310
Clinical features of nephrotic syndrome?
Protein in urine so low plasma albumin resulting in oedema (periorbital, scrotal, vulval, leg and ankle) May have ascites May have breathing difficulties resulting in breathlessness
311
Complications of nephrotic syndrome?
infection - peritonitis - cellulitis Hypovolaemia VTE - PE Acute renal failure protein depletion
312
Most common cause of nephrotic syndrome in kids
Minimal change glomerulonephritis. Epithelial cell foot processes fuse
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What is steroid sensitive nephrotic syndrome? What are the features that suggest this?
When the nephrotic syndrome is treatable with steroids, normally: Between 1-10years No macroscopic haematuria Normal BP Normal complement Normal renal function
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Management of nephrotic syndrome
Normally try steroids Try to use steroid sparing agents as many will relapse and steroid over use can cause all kinds of shit Prevent complications: Maintain hydration - Don't restrict fluids (unless must) - IV albumin - Caution with diuretics Minimise oedema - Low salt Prevent infection - Imms If they have lots of relapses then will need steroids long term
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If the urine is brown or red what is the haematuria likely caused by?
Brown - glomerular Red - LUTI
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Most common cause of haematuria?
UTI
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Causes of acute nephritis in children?
Post infective (strep) - doesn't last long, prognosis is good Vasculitis e.g. HSP IgA nephropathy
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Features of acute nephritis in children?
Decreased urine output and volume overload Hypertension Oedema Haematuria and proteinuria
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Management of acute nephritis in children?
Manage fluid and electrolyte balance, diuretics when necessary.
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Definition of HTN in kids?
Blood pressure above the 95th centile for height, age and sex
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Presentation of HTN in kids?
Vomiting, headaches facial palsy, retinopathy, convulsions or proteinuria.
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Causes of hypertension in kids?
Renal Polycystic kidneys, tumours, parenchymal disease Coarctation of the aorta Catecholamine excess - Phaeochromocytoma Endocrine - CAH - Cushings - Hyperthyroidism Essential HTN
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Meningococcal septicaemia most likely causative organism?
Neisseria meningitidis - gram negative
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Presentation of bacterial meningitis and of meningococcal septicaemia?
Different conditions, bacterial meningitis, does often present with sepsis though: Bacterial meningitis - Fever - Headache - Stiff neck, bulging fontanelle, photophobia - Altered mental state - Non-blanching rash Shock Seizures, focal neurological signs Meningococcal septicaemia without meningitis probably wouldn't have the classic stiff neck/photophobia/bulging fontanelle
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Red flags of SOL in kids?
Visual field deficits Cranial nerve abnormalities Headache worse on lying down, wakes kid up associated with morning nausea or vomiting Abnormal gait Torticolitis (tilting of the head) Growth failure papilloedema (late) Cranial bruits Personality change
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Three main divisions of headache in kids?
Primary - Migraine - Tension - Cluster - Cough/exertional Secondary - Due to SOL or raised ICP Trigeminal or other neuralgia
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What is the most common migraine in kids?
Migraine without aura (90%)
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Features of migraine without aura in kids? How does migraine with aura differ?
1-72hrs Commonly bilateral, may be unilateral though Pulsatile, normally over temporal or frontal area Photophobia/phonophobia GI symptoms such as nausea, vomiting, abdo pain Aggravated by physical activity Aura obviously also has aura, this may be visual sensory or motor and positive or negative symptoms
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Management of headaches (primary)?
Rescue - Analgesia (paracetamol and NSAIDS) - Antiemetics - 5HT agonists Prophylaxis - Pizofen (5HT antagonists) - Beta-blockers - Sodium channel blockers Psychosocial support
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Most likely pathogen to cause encephalitis in children?
Herpes simplex - it is usually viral
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Presentation of encephalitis?
Most patients with viral encephalitis present with the symptoms of meningitis (fever, headache, neck stiffness, vomiting) followed by altered consciousness, convulsions, and sometimes focal neurological signs, signs of raised intracranial pressure, or psychiatric symptoms. There may be an association with a history of infection elsewhere in the body.
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BDZ in status epilepticus in children?
Rectal diazepam
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How do you calculate maintenance fluids in kids?
first 10kg - 1 Litre (100ml/kg) 10-20kg - 500ml (50ml/kg) 20-80kg 20ml per kg
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What is neurofibromatosis type 1 and type 2?
Genetic disorder causing lesions to the skin, nervous system and skeleton Type 1 - More common - Genetic inheritance - autosomal dominant - cafe au lait spots - increased risk of developing benign and malignant tumours Type 2 - less common - presents in adolescence - Central CNS tumours e.g. vestibular schwannoma
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Diagnostic criteria for NF1 diagnosis?
Need two or more of these: Six or more cafe au lait spots >5mm (>15mm after puberty) Axillary freckles >1 neurofibroma optic glioma Lisch nodule (Iris haemotoma) Sphenoid dysplasia First degree realtive
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What is tuberous sclerosis?
genetic disorder resulting in the growth of benign tunours around the body, may have neurocutaneous and systemic lesions
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What is haemophilia, what are the types, inheritance pattern and features?
Haemopilia A and B, X-linked recessive disorders In A there is factor 8 deficiency and is more common B is factor 9 deficiency, less common 2/3rd of patients have family history, 1/3rd don't as can be a de novo mutation. Can range from mild to severe deficiency: - severe would have recurrent spontaneous bleeding into joints and muscles - would lead to bad arthritis normally present <1 year, can lead to intraventricular haemorrhage in neonates and bleeding episodes (into joints and stuff when starting to walk)
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Management of Haemophilia?
Replace the lost factor (8 in A, 9 in B), prophylactic therapy normally begins at 2-3 years.
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What is ITP?
Immune thrombocytopenic purpura - lack of circulating platelets leading to bleeding Normally occurs in children following viral infection or immunisation and is self-limiting in 6-8 weeks. Can cause bleeding and purpura
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Management of sickle cell disease?
Full imms and antibiotic prophylaxis (due to increased risk of infection) throughout childhood. Supportive in acute event Hydroxyurea may predict in children who are struggling
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Clinical manifestations of Sickle cell disease?
Anaemia, chronic and acute Infection Painful crisis Priaprism - needs to be treated promptly Splenomegaly Other long term issues
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Main causes of iron deficiency in children?
Main: - Inadequate intake (may be because still breast fed >6 months) - Malabsorption - Blood loss Other disorders such as: - Sickle cell - Thalassaemias - G6PD deficiency, spherocitosis - Haemophilias
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Most common cancer in children? how o you differentiate ALL and AML
ALL AML and ALL are diferentiated morphologically
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Leukaemia presentation, features and management?
2-5 years, normally insidious over a few weeks, but can be v rapid. General: Malaise, anorexia Bone marrow infiltration - Anaemia - pallor, lethargy - Netropaenia - infection - thrombocytopaenia - bruising, petechiae - bone pain hepatosplenomegaly, lymphadenopathy Other: - CNS - headache. vomiting Testicular enlargement
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Types of lymphoma in childhood?
Hodkin and non-hodgkin hodgkin is painless lymphadenopathy, non is mediastinal mass, prognosis is good with combined chemotherapy
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What is CVID?
B cell issue umbrella diagnosis in that it encompasses a group of genetic disorders which result primarily in hypogammaglobulinaemia or failure of antibody production Presents with recurrent infection
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What is anaphylaxis and how do you manage it?
Severe rapid onset immunological reaction that threatens ABC Normally IgE mediated reaction to food allergy, asthma is a. risk factor. ABCDE approach High flow O2 Adrenaline IM: >12 500mg (0.5ml of 1 in 1000) 6-12 0.3ml <6 0.15ml Fluid and steroids
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Orbital cellutitis and periorbital cellulitis differences and management?
Periorbital cellulitis is not in the obit yet, it presents with erythema, tenderness and oedema of the eyelid, it may follow local trauma. Needs prompt Iv antibiotics to prevent it spreading to the orbit and becoming orbital cellulitis. Orbital cellulitis is an emergency, there is proptosis, painful and limited ocular movement and reduced visual acuity. It may be complicated by meningitis, cavernous sinus thrombosis or abscess formation.
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Chickenpox presentation and management?
Varicella zoster infection, normally happens by 5 years Pyrexia Headache and malaise Vesicles for 3-5 days - papule, vesicle, pustule and crust Can be very itchy, less so in younger kids Not a problem in younger kids but much more of a problem in older adults if they haven't had it. Just general supportive management, can use antihistamine and paracetamol
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What is slapped cheek disease? Presentation and management?
Fith disease or parovirus infection or erythema infectiosum. Coryzal symptoms at first then free from symptoms for 7-10 days Then slapped cheek rash About 1-4 days after the facial rash appears, an erythematous macular rash develops on the extremities, mainly on the extensor surfaces. disappears after 3-21 days.
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What is hand foot and mouth disease, how does it present and how is it managed?
Viral illness which commonly causes lesions involving the mouth, hands and feet. Can affect other areas such as genitalia and buttocks. Coxsackievirus A16 (CA16) and enterovirus 71 (EV71) Prodrome such as low grade fever, malaise and loss of apetite Lesions begin in mouth, macular that progress to ulcers, then skin lesions Self-limiting Management is supportive, assure it is not foot and mouth disease in animals. Paracetamol, soft diet and analgesics. May rarely have complications such as meningitis or encephalitis, infection from open sores, cardiorespiratory failure.
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What is JIA?
Juvenile idiopathic arthritis (JIA) is defined as joint inflammation presenting in children under the age of 16 years and persisting for at least six weeks, with other causes excluded. Lots of different types, can cause fever, is not infective in origin.
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What is malrotation and volvulus?
A volvulus is a complete twisting of a loop of intestine around its mesenteric attachment site. This can occur at various locations of the gastrointestinal (GI) tract, including the stomach, small intestine, caecum, transverse colon and sigmoid colon. Midgut malrotation refers to twisting of the entire midgut about the axis of the superior mesenteric artery (SMA). Bilious vomiting is key, malrotation may lead to volvulus, which is pretty bad Volvulus may present with abdo mass, distention and pain - urgent care needed, surgical correction.
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Appendicitis presentation?
RIF (may start periumbilical) pain, worse on movement is key, nausea vomiting and anorexia. Rebound tenderness
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What causes roseola infantum?
Human Herpes virus 6
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Most likely cause of nephrotic syndrome in children/young adults?
Minimal change disease