Children's Health Flashcards
1
Q
Recall the 5 age ranges that paediatric patients are divided into
A
- Neonate - Birth to 1 month
- Infant - 1 Month to 2 years
- Young child - 2 to 6 yeas
- Child - 6 to 12 years
- Adolescent - 12-18 years
2
Q
Why is the use of closed questions even less reliable when working with children?
A
Children will often say yes to closed questions with the intention of pleasing the examiner
3
Q
An infant should have received all of their vaccinations for rotavirus by what age?
A
12 weeks
4
Q
At the age of 2 months what hearing, language and communication milestones would the infant be expected to have reached? (2)
A
- Coo’s and gurgling sounds
2. Turns head towards sounds
5
Q
At the age of 8 months what hearing, language and communication milestones would the infant be expected to have reached? (2)
A
- Understands ‘No’
2. Mamma and Dadda sounds
6
Q
At the age of 18 months what hearing, language and communication milestones would the infant be expected to have reached? (2)
A
- Can say 10 words
2. Says No and shakes head
7
Q
At 3 years of age what hearing, language and communication milestones would the child be expected to have reached? (2)
A
- Understand words such as in/on/under
2. Carries on a conversation using 2-3 sentences
8
Q
At the age of 2 months what social and emotional milestones would the infant be expected to have reached? (3)
A
- Begins to smile at people
- Tries to look at parents
- Sucks on hand to self soothe
9
Q
At the age of 8 months what social and emotional milestones would the infant be expected to have reached? (2)
A
- Stranger awareness
2. Has a favourite toy
10
Q
At the age of 18 months what social and emotional milestones would the infant be expected to have reached? (2)
A
- Temper tantrums
2. Points to show something interesting
11
Q
At 3 years of age what social and emotional milestones would the infant be expected to have reached? (3)
A
- Copies adults and friends
- Takes turns playing games
- Shows concern for friends crying
12
Q
At the age of 2 months what cognitive and fine motor milestones would the infant be expected to have reached? (2)
A
- Begins to following things with eyes
2. Begins to act bored
13
Q
At the age of 8 months what cognitive and fine motor milestones would the infant be expected to have reached? (3)
A
- Transfers from one hand to another
- Picks up cereal between thumb and index finger
- Plays peek a boo
14
Q
At the age of 18 months what cognitive and fine motor milestones would the infant be expected to have reached? (2)
A
- Follow one step verbal commands
2. Scribble on own
15
Q
At 3 years of age what cognitive and fine motor milestones would the infant be expected to have reached? (2)
A
- Does jigsaw of 3-4 pieces
2. Copies a circle with a pencil
16
Q
Recall the normal ranges of a 1 minute respiratory rate in each of the 5 paediatric age ranges
A
- Neonatal: 30-39
- Infant: 25-34
- Young Child: 20-29
- Child: 20-29
- Adolescent: 15-24
17
Q
Recall the normal ranges for heart rate in each of the 5 paediatric age ranges
A
- Neonatal: 110-159
- Infant: 100-149
- Young child: 80-119
- Child: 70-119
- Adolescent: 65-99
18
Q
Recall the normal ranges for systolic blood pressure in each of the 5 paediatric age ranges
A
- Neonatal: 60 and above
- Infant: 70 and above
- Young child: 85 and above
- Child: 90 and above
- Adolescent: 90 and above
19
Q
What is the most common cause of congenital cataracts?
A
Intrauterine infections e.g. rubella
20
Q
On examination of a neonate, what is the top differential to consider when you observe asymmetrical thigh creases?
A
Unilateral dislocation of the hip
21
Q
During paediatric CPR, which vessels should be used to check for circulation? (2)
A
- Infant - Brachial or femoral
2. Child - Carotid or femoral
22
Q
What is the CPR ratio protocol for a child or infant?
A
- Initiate with 5 rescue breaths
- Complete 15 chest compressions
- Continue cycles of CPR at a 2:15 ratio
23
Q
What is the most likely cause of cardiac arrest in children?
A
Asphyxiation
24
Q
What part of the physical examination is contra-indicated when examining a child with a suspected upper airway obstruction?
A
Examination of the throat as this risks exacerbating the obstruction
25
Suggest 6 possible differential diagnoses for an infant presenting with stridor
1. Viral croup
2. Epiglottitis
3. Foreign body aspiration
4. Anaphylaxis
5. Bacterial tracheitis
6. Laryngomalacia
26
Define haemangioma
Benign vascular lesions that typically appear during the first weeks of life - seen as pink or blue macules / patches
27
What is the first line treatment for a haemangioma?
Systemic beta blockers e.g. propanolol
28
Describe the natural course of a haemangioma
Enlarges steadily over the first 12-24 months of life
29
What are the 5 principle clinical features associated with mucopolysaccharidoses?
1. Hepatosplenomegaly
2. Neurodevelopmental delay
3. Cardiorespiratory disease
4. Joint contractures
5. Failure to thrive
30
Mucopolysaccharidoses is a form of what type of metabolic disorder?
Hereditary lysosomal storage disease
31
The red flag symptom: 'Bile stained vomit' in the scenario of a vomiting child is indicative of what condition?
Intestinal obstruction
32
The red flag symptom: 'Haematemesis' in the scenario of a vomiting child could be indicative of which condition? (3)
1. Oesophagitis
2. Peptic ulceration
3. Oral/ nasal bleeding
33
The red flag symptom: 'Projectile vomiting in the first weeks of life' in the scenario of a vomiting child could be indicative of which condition?
Pyloric stenosis
34
The red flag symptom: 'Vomiting at the end of paroxysmal coughing' in the scenario of a vomiting child could be indicative of which condition?
Whooping cough (pertussis)
35
The red flag symptom: 'Blood in the stool' in the scenario of a vomiting child could be indicative of which condition? (2)
1. Intussusception
| 2. Gastroenteritis - Salmonella or Campylobacter
36
What is Intussusception?
The prolapse of one part of the intestine into the lumen of a distal adjoining section which leads to intestinal obstruction in infants between 3 and 12 months
37
The red flag symptom: 'Bulging fontanelle or seizures' in the scenario of a vomiting child could be indicative of which condition?
Raised intracranial pressure
38
Give 5 potential complications of gastro-oesophageal reflux in infants
1. Failure to thrive due to severe vomiting
2. Oesophagitis (may also be coupled with an iron deficiency anemia)
3. Recurrent pulmonary aspirations
4. Dystonic neck posturing (Sandifer syndrome)
5. Apparent life threatening events (ALTE)
39
Name a H2- receptor anatagonist that can be used in the treatment of GORD
Ranitidine
40
Name a drug that can be used to enhance gastric emptying
Domperidone
41
Give 3 clinical signs associated with pyloric stenosis
1. Visible gastric peristalsis
2. Palpable abdominal mass
3. Dehydration
42
What is the typical age of onset of croup?
6 months - 3 years
43
What dose of adrenaline is used in the immediate management of moderate to severe croup in the hospital setting?
Nebulised adrenaline 5mls 1:1000
44
What dose of oral steroids should be given in the management of severe croup?
Single dose of dexamethasone 0.15mg/kg (can be repeated 12 hours later)
45
The term anaphylaxis describes what clinical state? (3)
Hypotension, Bronchoconstriction and/or airway obstruction
46
What 4 questions must you ask when assessing a child's risk of future anaphylactic reactions?
1. Does the child have asthma?
2. If they have asthma, what treatment to they receive?
3. Do they take a regular preventer inhaler?
4. Before the initial reaction, how much of the allergen/ foodstuff had they been in contact with?
47
Define type II hypersensitivity
Cytotoxic, antibody dependent hypersensitivity
48
Define type III hypersensitivity
Immune complex disease caused by IgG
49
Define type IV hypersensitivity
T cell mediated delayed hypersensitivity. (Antibody independent)
50
Name the 2 immune mediators that are released by Mast cells
1. Prostoglandins
| 2. Histamine
51
What are the 4 main physiological actions of histamine?
1. Localised irritation
2. Endothelial cell separation
3. Bronchoconstriction
4. Vasodilation
52
Outline the 6 criteria to consider when prescribing an adrenaline pen
1. History of anaphylaxis
2. Previous cardiovascular/ respiratory involvement
3. Evidence of airway obstruction
4. Poorly controlled asthma requiring regular inhaled corticosteriods
5. Reaction to a small amount of allergen
6. Ease of allergen avoidance
53
Outline the characteristics of a 'spontaneous effective cough' in a child with a foreign body aspiration (4)
1. Crying or verbal response to questions
2. Loud cough
3. Able to take a breath before coughing
4. Fully responsive
54
What intervention should be administered to a conscious child who is choking and exhibits an 'ineffective cough' ?
Cycles of 5 back blows followed by 5 thrusts
55
What is another term for croup?
Laryngotracheobronchitis
56
What is the most common causative organism of croup?
Parainfluenza virus
57
What age group are at risk of developing croup?
Children under the age of 6
58
Recall the 'mediator' implicated in each of the 4 classifications of hypersensitivity reaction
Type I - IgE
Type 2 - IgM
Type 3 - IgM
Type 4 - T cells
59
A penicillin allergy is an example of which type of hypersensitivity reaction?
Type 2 - IgM mediated
60
Serum sickness ( in response to the therapeutic use of anti-venom) is an example of which type of hypersensitivity reaction?
Type 3 - IgM mediated
61
Outline 4 potential symptoms that are caused by the deposition of antigen- antibody complexes during a type III hypersensitivity reaction
1. Fever
2. Vasculitis
3. Arthritis
4. Nephritis
62
What are the most common inhaled foreign objects seen in children?
Nuts and seeds
63
What is the most common birth defect?
Congenital heart disease
64
Are left to right shunts cyanotic or acyanotic CHD's?
Acyanotic
65
What is Eisenmenger's physiology?
The evolution of an acyanotic CHD to a cyanotic lesion as a result of pulmonary vascular obstructive disease. (i.e. shunt reversal).
66
Recall the 5 examples of left to right shunting congenital heart lesions
1. Ventricular septal defect (VSD)
2. Atrial septal defect (ASD)
3. Atrioventricular septal defect (AVSD)
4. Patent ductus arteriosus (PDA)
5. Partial anomalous pulmonary venous connection (PAPVC)
67
Name 2 obstructive valvular lesions that are seen as congenital heart defects
1. Pulmonary valve stenosis
| 2. Aortic valve stenosis
68
Name 2 obstructive non-valvular lesions that are seen as congenital birth defects
1. Left ventricular outflow tract obstruction
| 2. Coarctation of the aorta
69
What is the most common form of congenital heart defect?
Ventricular septal defect
70
List 4 subtypes of ventricular septal defect
1. Perimebranous
2. Outlet
3. Inlet
4. Muscular
71
Suggest 5 clinical signs which may indicate excess pulmonary blood flow in a newborn
1. Tachypnoea
2. Tachycardia
3. Pallor
4. Poor feeding
5. Poor weight gain
72
During a CV examination what are the 3 principle indicators of a VSD?
1. Low to mid frequency pansystolic murmur
2. Prominent precordial impulse
3. Loud/single second heart sound ( in those with pulmonary hypertension)
73
What characteristics of a VSD may be visible on CXR? (2)
1. Cardiac enlargement
| 2. Increased pulmonary vascular markings
74
Are atrial septal defects more common in male or female newborns?
Female to male predominance of 2:1
75
What is the most common subtype of atrial septal defect?
Ostium secundum defect
76
In what area does an ostium primum atrial septal defect occur?
In the inferior aspect of the atrial septum
77
Name the 4 subtypes of atrial septal defect
1. Ostium secundum
2. Ostium primum
3. Sinus venosus
4. Unroofed coronary sinus
78
What are the 4 main clinical characteristics of an undiagnosed ASD that only becomes symptomatic later in life?
1. Exercise intolerance
2. Atrial arrhythmia
3. Increased pulmonary blood flow
4. Pulmonary vascular obstructive disease (Eisenmenger's Syndrome)
79
What form of congenital heart disease is found in around ~40% of individuals with Down's syndrome?
Atrioventricular septal defect (Endocardial cushion defect)
80
What is the incidence of a patent ductus arteriosus in full term infants?
Occurs in 1 in 5000 live births
81
Give 2 circumstances that increase the likelihood of a newborn having a patent ductus arteriosus
1. Pre-term
| 2. Born at high altitude
82
Name 2 medications that can be used to close a patent ductus arteriosus in pre-term infants
1. Ibuprofen
| 2. Indomethacin
83
What is Scimitar syndrome?
Syndrome characterised by anomalous venous return from the right lung into the systemic venous drainage instead of directly into the left atrium
84
Outline the 4 abnormalities that make up the Tetralogy of Fallot
1. Outlet ventricular septal defect
2. Right ventricular outflow tract obstruction
3. Overriding aorta
4. Right ventricular hypertrophy
85
What compound can be used to maintain the patency of the ductus arteriosus?
Prostaglandin E1
86
Name 3 anomalies that have an association with truncus arteriosus
1. DiGeorge Syndrome
2. Interrupted aortic arch
3. Truncal valve insufficiency
87
Define Ebstein's anomaly
Malformation of the tricuspid valve - delamination of the valve leaflet leads to severe valvular regurgitation
88
Broadly outline hypoplastic left heart syndrome
Spectrum of lesions that consist of an intact ventricular septum with under development of the left ventricle, mitral and aortic valves
89
Approximately what percentage of the general population have a bicuspid aortic valve?
2%
90
What form of congenital heart abnormality is associated with William's syndrome?
Supravalvular aortic stenosis
91
Aortic coarctation has an association with which genetic condition in females?
Turner's syndrome
92
Pulmonary valve stenosis has the strongest association with which genetic syndrome?
Noonan syndrome
93
Up to one month of age, what is the minimum milk requirement in order to provide the infant with enough calories to grow?
150 mls/kg/day
94
Give 3 complications that have been linked with an excess maternal sugar intake
1. Increased risk of gestational diabetes
2. Preeclampsia
3. Pre-term birth
95
Give 2 complications associated with prolonged rupture of the membranes during labour
1. Oligohydramnios
| 2. Increased incidence of neonatal infection
96
Which 9 diseases does the the newborn blood spot (heel prick) test screen for?
1. Sickle cell disease
2. Cystic fibrosis
3. Congenital hypothyroidism
4. Inherited metabolic diseases (6)
97
Recall the inherited metabolic diseases that are tested for with the newborn blood spot test (6)
1. Phenylketonuria (PKU)
2. medium chain acetyl-CoA dehydrogenase deficiency (MCADD)
3. Maple syrup urine disease (MSUD)
4. Isovaleric acidemia (IVA)
5. Glutaric aciduria type 1 (GA1)
6. homocystinuria (HCU)
98
Compare the relative congenital risk of gestational and pregestational diabetes
Only pregestational diabetes confers an increased risk of the foetus developing a congenital abnormality. Gestational diabetes offers no additional risk of congenital abnormality
99
Outline the 8 principle factors to consider when assessing an unwell infant from the end of the bed (8)
1. General dysmorphism
2. Neurological status
3. Colour
4. Any extra noises? e.g. Grunting, stridor, wheeze or crying
5. Signs of pain
6. Any obvious marks or rashes
7. Any secretions - eyes, nose or mouth
8. Any signs of increased work of breathing
100
During auscultation of a newborn , listening over which area will accentuate the murmur of a PDA?
Listen over the left scapula
101
What is a Mongolian blue spot?
A form of benign congenital birth mark - more prevalent in babies of colour
102
Suggest 6 potential medical causes of increased sleepiness in an infant
1. Hypoglycaemia - due to poor feeding
2. Exhausted - due to work of breathing and feeding
3. Encephalopathic due to infection
4. Encephalopathic due to ammonia build up secondary to a metabolic condition
5. Neurological secondary to head injury
6. Hypoxia
103
Name the 2 congenital heart abnormalities that can result in a 'small heart size' on CXR
1. Tetralogy of Fallot
| 2. TAPVD
104
Name the 2 congenital heart abnormalities that are NOT expected to alter the size of the heart on CXR
1. TGA
| 2. Pulmonary atresia
105
What type of murmur is associated with a VSD?
Holosytolic murmur
106
What is the most likely section in which a VSD may be found
Along the length of the membranous septum (superior section of the ventricular septum)
107
Over what age does oedema become part of the clinical presentation of heart failure?
2-3 years of age. Children younger than this may present with heart failure in the absense of pulmonary oedema
108
Why are the ACE inhibitors Captopril and Enalapril the most commonly used in paediatrics?
They are shorter acting than other ACE inhibitors
109
The embryological 'cardiac tube' is formed from what cell type?
Mesenchymal cells from the splanchnic mesoderm
110
What are the 2 main stages of cardiac tube development?
1. Looping
| 2. Septation
111
Name 2 acquired heart conditions that are seen in children
1. Myocarditis
| 2. Rheumatic heart disease
112
What are the 2 different means of forming a antenatal diagnosis of a congenital heart disease?
1. Routine scan
| 2. Family Hx of 1st degree relative
113
What are the examination findings on palpation and auscultation of an individual with a PDA? (2)
1. Bounding pulse
| 2. Continuous (systolic and diastolic) 'machinery-like' murmur
114
Name 2 other congenital abnormalities that have an association with coarctation of the aorta
1. Bicuspid aortic valve
| 2. VSD
115
Give 2 examination findings commonly associated with coarctation of the aorta
1. Weak or absent femoral pulses
| 2. Systemic hypertension of the upper limbs
116
Coarctation of the aorta leaves newborns at an increased risk of developing which medical condition?
Necrotising enterocolitis - as a result of a reduced blood supply to the gut
117
List the 4 main features of the clinical presentation of an infant with Fallot's tetralogy
1. Central cyanosis
2. Pulmonary oligaemia on CXR
3. Hypercyanotic episodes
4. Loud ejection systolic murmur ( due to pulmonary stenosis)
118
Name 2 chromosome abnormalities that have an association with coarctation of the aorta
1. Turner's syndrome
| 2. DiGeorge's syndrome (22q11 deletion)
119
Name 5 genetic conditions that have an association with some form of congenital heart defect
1. Trisomy 13 - Patau syndrome
2. Trisomy 18 - Edward syndrome
3. Turner's syndrome
4. Kartagener's syndrome
5. DiGeorge's syndrome
120
Recall 5 clinical signs associated with Down's syndrome
1. Hypotonic
2. Flat occiput
3. Single palmar creases
4. Incurved fifth finger
5. Wide 'sandal gap' between the big and second toe
121
Give 3 medical conditions for which children with Down's syndrome have an increased risk of developing
1. Hypothyroidism
2. Vision and/or hearing loss
3. Atlanto-axial instability
122
What is the most common cytogenetic causal mechanism of trisomy 21?
Meiotic non disjunction
123
Name 2 gastrointestinal anomalies that have a higher incidence in patients with Down's syndrome
1. Duodenal atresia
| 2. Hirschsprung disease
124
Normally when is the ductus arteriosus expected to close?
~ 3 days after birth
125
Name the 6 most commonly implicated viruses in childhood respiratory tract infections
1. Respiratory syncytial virus
2. Rhinovirus
3. Parainfluenza
4. Influenza
5. Metapneumovirus
6. Adenoviruses
126
Give 4 types of upper respiratory tract infection seen in children
1. Common cold (coryza)
2. Sore throat (pharyngitis - including tonsillitis)
3. Acute otitis media
4. Sinusitis
127
Describe the clinical sign Harrison's sulci and give 2 differentials that it may indicate
Groove at the lower edge of the rib margin - seen when the lower chest is drawn in. Can be seen in:
1. Infants with abnormally weak bones e.g. Rickets
2. Chronic respiratory disease e.g. severe asthma
128
According to the british thoracic society guidelines on the management of pneumonia in children, the presence of which 3 clinical features is sufficient for a working diagnosis of bacterial pneumonia?
1. Persistent or repetitive fever >38.5
2. Chest recession
3. Raised respiratory rate
129
Oxygen therapy should be considered for childhood pneumonia if saturations fall below what level?
< 92 Sp02
130
What is the first line antibiotic used in the treatment of community acquired pneumonia in children?
Amoxicillin
131
What additional therapy should be offered to a child with community acquired pneumonia not responding to first line empirical therapy?
Macrolide antibiotic
132
CAP in children caused by either mycoplasma or chlamydia should be treated with which antibiotics?
Macrolide antibiotic
133
CAP in children caused by influenza should be treated with which antibiotic?
Co-amoxiclav
134
Give 3 appropriate investigations for a child presenting with recurrent lower respiratory tract infections and possible bronchiectasis
1. Sweat chlorine test - to rule out CF
2. CT scan of the chest
3. Measure serum immunoglobulin levels
135
What is the inheritance pattern of cystic fibrosis?
Autosomal recessive
136
Where does the mutation for cystic fibrosis occur?
On the long arm of chromosome 7 - this results in abnormal synthesis of the cystic fibrosis transmembrane regulator protein (a form of chloride channel).
137
Outline the 4 components of the 'classical' presentation of a patient with CF
1. Child aged 0-2
2. Recurrent chest infections
3. Large offensive stools
4. Failure to thrive
138
Name 3 organisms for which CF patients have a predisposition for developing chronic infections of
1. S.Aureus
2. H. Influenza
3. Pseudomonas Aeruginosa
139
Name a prophylactic oral antibiotic that can be used in the management of CF
Flucloxacillin
140
List 7 organs that can be affected by cystic fibrosis
1. Sinuses - sinusitis
2. Lungs
3. Skin
4. Pancreas - blocked pancreatic ducts
5. Liver - blocked biliary ducts
6. Intestines - cannot fully absorb nutrients
7. Reproductive organs - infertility in men (due to absence of vas deferens) and subfertility in females
141
What is Mounier-Kuhn syndrome?
A rare condition characterised by tracheobronchial dilatation and recurrent lower respiratory tract infections
142
What are cromones and give 2 examples
Form of anti-allergic medication - act as mast cell stabilisers i.e. to reduce severity of an allergic response. Examples of this drug class include: Cromoglicate and nedocromil
143
List 5 examples of non atopic (intrinsic) asthma exacerbators
1. Stress
2. Exercise
3. Occupational exposure
4. Aspirin
5. Cold
144
Name the 3 layers that make up the mucosa of the respiratory tree
1. Epithelium
2. Basement membrane
3. Lamina Propria
145
Compare the relative levels of eosinophils present in asthma and chronic bronchitis
Elevated levels of eosinophils seen in asthma. Chronic bronchitis has no affect on eosinophil production
146
Which cell type is responsible for the the late phase reaction as part of the pathophysiology of asthma?
Eosinophils
147
Why does status asthmaticus not respond to convential atopic asthma therapy with bronchodilators and corticosteriods?
The allergic reaction ( in the case of atopic asthma) is now mediated by eosinophil activation is part of the late stage reaction - thus it is now independent of the initial inhaled allergen.
148
Broadly what is the pathophysiology of aspirin related bronchospasm (a form of intrinsic asthma)?
Aspirins inhibition of the cyclooxygenase pathway can lead to compensatory over stimulation of the lipooygenase pathway and release of leukotrienes which is sufficient to induce bronchospasm
149
Give 2 microscopic findings that can be found on examination of the airways of a known asthmatic
1. Curschmann spirals
| 2. Charcot- Layden crystals
150
What is the role of bronchial thermoplasty in the treatment of asthma?
Heat from therapeutic radio-waves can be used to reduce the thickness of the smooth muscle of the airways in patients with severe asthma
151
A child with asthma should be trialed with a metred dose inhaler at ~ what age?
~ Aged 12. Children under this age tend to lack sufficient coordination and thus should be prescribed a spacer instead
152
What is the most common genetic mutation associated with cystic fibrosis seen in the UK?
Delta F508 mutation
153
An infant with ? Shaken baby syndrome should receive which investigations? (5)
1. Head CT
2. Skeletal survey
3. Ophthalmology review
4. Metabolic testing
5. Detailed testing of potential blood coagulation disorders
154
Name 5 viruses that have been linked with viral aplastic anemia
1. Hepatitis
2. Cytomegalovirus
3. HIV
4. Parvovirus B19
5. Epstein- Barr virus
155
Name the 5 types of child maltreatment
1. Physical
2. Neglect
3. Emotional
4. Sexual
5. Factitious or induced injury
156
Give 8 features which may be displayed by infants with a non accidental head injury
1. Irritability
2. Poor feeding
3. Increasing head circumference
4. Seizures
5. Reduced GCS
6. Bulging fontanelle
7. Anemia
8. Retinal haemorrhage
157
Define Job's syndrome
Hyper IgE Syndrome is an immune deficiency caused by a genetic mutation in either STAT 3 or DOCK8
158
Recall the components of the 'toxic trio' with reference to child safeguarding
1. Domestic violence/ abuse
2. Parental mental health
3. Parental substance abuse
159
Suggest 6 medical differentials for non accidental injury in a child
1. Mongolian Blue spots
2. Bleeding disorders
3. Osteogenesis imperfecta
4. Rickets
5. Impetigo
6. Staph scalded skin syndrome
160
What is Pierre Robin syndrome?
A congenital defect that results in a characteristic series of facial abnormalities
161
What are the 3 main features of Pierre Robin syndrome?
1. Micrognathia
2. Glossoptosis
3. Upper airway obstruction
162
What are the 3 main characteristics of an accidental scald injury in a child?
1. Spares the flexures
2. Asymmetrical
3. Clear splash pattern to injury
163
Suggest 9 potential differential diagnoses for a child presenting with abdominal pain
1. Constipation
2. Acute appendicitis
3. Gastroenteritis
4. UTI
5. Abdominal trauma
6. Cholelithiasis/ cholecystitis
7. Primary dysmenorrhea
8. Pneumonia
9. Functional abdominal pain
164
Name 2 conditions that can potential give rise to secondary Intussusception
1. HSP - Henoch-Schonlein Purpura
| 2. Lymphoma
165
What is the most frequently seen malformation of the Gi tract?
Meckel's diverticulum
166
What is the most common cause of haemoptysis in children?
Mallery-Weiss tear
167
Give 5 red flag symptoms when assessing a child with abdominal pain
1. Child aged < 5
2. Weight loss
3. Blood in the stool
4. Poor growth
5. Symptoms regularly waking child from sleep
168
What are the 5 most common causes if malaena in children>
1. Bacterial diarrhoea
2. Inflammatory bowel disease
3. Tear of anal vein
4. Polyps
5. Intussusception
169
What does the phrase 'cobblestoning' refer to with reference to Crohn's disease?
Longitudinal ulcers with intervening oedematous mucosa found along the length of the affected portion of the GI lumen
170
Give 4 complications associated with inflammatory bowel disease
1. Toxic megacolon
2. Haemorrhage
3. Stricture
4. Malignancy
171
Give 3 risk factors for the development of colon cancer in patients with inflammatory bowel disease
1. Frequent flares
2. Long standing disease
3. PSC - Primary sclerosing cholangitis
172
TTG antibody used as a screening tool for which condition?
Coeliac disease
173
What is the most likely causative organism of haemolytic uraemic syndrome?
E.Coli O169
174
What is the definition of anorexia nervosa?
Eating disorder characterised by reducing calorie intake leading to a low body weight, an intense fear of gaining weight and body image disturbances
175
Recall 4 risk factors associated with anorexia nervosa according to the BMJ
1. Female sex
2. Adolescence and puberty
3. Obsessive and perfectionist traits
4. Exposure to western media
176
Outline 4 1st line investigations for a patient presenting with ? Anorexia Nervosa
1. FBC
2. Serum chemistry
3. Thyroid function tests
4. Liver function tests
177
Give 4 risks associated with rapid weight loss
1. Refeeding syndrome
2. Hypoglycaemia
3. Increased risk of infection
4. Cardiac arthymias
178
Give 10 causes of rapid weight loss in adolescent
1. Coeliac disease
2. Type 1 diabetes
3. Hyperthyroidism
4. Malignancy
5. Anorexia Nervosa
6. Inflammatory bowel disease
7. Oesophageal problems e.g. achalasia
8. Severe depression/OCD/ autism
9. Juvenile arthritis
10. Addison's
179
What causes Lanugo hair growth?
Response to a the loss of the insulating effect of fat tissue in patients with Anorexia Nervosa
180
In a patient presenting with anorexia, what weight for height ratio would be an indication for the commencement of vitamin supplementation?
< 75%
181
How can a patient's treatment for anorexia be monitored for potential refeeding syndrome?
Close monitoring of serum phosphate levels - a drop in phosphate may indicate refeeding syndrome.
182
Suggest a medication that can be used in the treatment of OCD
Fluoxetine
183
What are the 3 main types of eating disorder seen in young people?
1. Anorexia Nervosa
2. Bulimia Nervosa
3. EDNOS - eating disorder not otherwise specified
184
Name 2 common co-morbidities associated with anorexia
1. Depression
| 2. Obsessive compulsive disorder
185
What are the 3 most significant mortality risks in patients with Anorexia Nervosa?
1. Sudden cardiac death
2. Suicide
3. Chronic emaciation + pneumonia
186
Beri Beri syndrome is caused by a deficiency of which vitamin
Thiamine - Vitamin B1
187
Give 5 physical features of dehydration seen in infants
1. Sunken anterior fontanelle
2. Dry mucous membranes
3. Tachycardia
4. Reduced capillary refill time
5. Reduced skin turgor
188
What are the 2 most common causes of gastroenteritis in young children?
1. Rotavirus
| 2. Adenovirus
189
Give 5 potential causes of blood in the stool associated withs severe diarrhoea and vomiting
1. Rotavirus
2. E. Coli
3. Campylobacter
4. Intussecpition
5. Shigella
190
What is the most common cause of acute renal failure in children?
Haemolytic uraemic syndrome
191
Projectile vomiting under 2 months of age is pathopneumonic for which medical condition?
Pyloric stenosis
192
What is the most common causative organism of food poisoning in the UK?
Campylobacter
193
Give 5 anti infective components of breast milk
1. Secretory IgA
2. Bifidus factor
3. Lysozyme
4. Lactoferrin
5. Interferon
194
Name 2 parasites that can cause gastroenteritis
1. Giardia lamblia
| 2. Cryptosporidium
195
Name 4 organisms that can cause watery stool in a presentation of gastroenteritis
1. Cholera
2. E. Coli
3. C. Diff
4. Cryptosporidium ( in HIV infections)
196
Give 6 red flag features of clinical dehydration that may be found on clinical examination
1. Altered responsiveness/ conscious level
2. Appears unwell/ deteriorating
3. Sunken eyes
4. Tachypnoea
5. Tachycardia
6. Reduced skin turgor
197
Characterise mild, moderate and severe dehydration by the % of bodyweight lost
Mild - <4%
Moderate - 4-6 %
Severe - >7%
198
Outline 5 factors that can increase an individuals risk of dehydration
1. Children under the age of 1, particularly those under 6 months of age
2. Low birth weight infants
3. More than 5 loose stools and 2 episodes of vomiting in the last 24hrs
4. Not offered or unable to tolerate replacement fluids on presentation
5. Malnourished children
199
Give 5 features of hypernatremia dehydration
1. Jittery movements
2. Increased muscle tone
3. Hyperreflexia
4. Convulsions
5. Drowsiness or coma
200
Give 4 indications for IV fluid treatment in a child presenting with diarrhoea and vomiting
1. Shock
2. Red flag symptoms/ signs of dehydration
3. Persistent vomiting
4. Hypoglycaemia
201
What is the fluid treatment for hypovolaemic shock in children?
Bolus 20 ml/kg of 0.9% NaCl by rapid Iv infusion. A second bolus may be given if shock persists
202
Recall the paediatric maintenance fluid values for each weight component of the fluid calculation
1. 0-10kg - 100 mls/kg
2. 10-20 kg - 50mls/kg
3. >20 kg - 20 mls/kg
203
Which antibiotic should be prescribed for the treatment of diarrhoea caused by Campylobacter?
Erythromycin
204
Which antibiotic should be prescribed for the treatment of diarrhoea caused by a C.Diff infection?
Metronidazole or Vancomycin
205
A 'right leg limping gait' accompanied by severe abdominal pain in a child is pathopneumonic of which medical condition?
Appendicitis
206
A diagnosis of Kawasaki's disease requires a high fever lasting more than 5 days alongside at least 4 of which 5 clinical findings?
1. Cervical lymphadenopathy
2. Bilateral non purulent conjunctival infection
3. Mucosal changes e.g. strawberry tongue/ cracked red lips
4. Red rash
5. Peripheral skin changes e.g. redness or oedema of the hands and/or feet
207
Suggest 5 conditions that may present with a similar rash to that associated with Kawasaki Disease
1. Toxic shock syndrome
2. Staph scalded skin
3. Scarlet fever
4. Measles
5. Juvenile idiopathic arthritis
208
Give 4 potential causes of a non blanching petechial/purpuric rash
1. Meningococcal septicaemia
2. Heinen Schonlein Purpura
3. Non accidental injury
4. Idiopathic thrombocytopenic purpura
209
What is the first line antibiotic therapy for suspects meningitis/ meningococcal septicaemia in the hospital setting?
Intravenous/ intraosseous cephalosporin
210
What is the normal age for the anterior fontanelle to close?
18-24 months
211
Give 6 tests that should be requested on a CSF sample from a patient with ? meningitis
1. Microscopy and gram stain
2. Culture and sensitivity
3. Glucose
4. Protein
5. Virology
6. PCR for virology, pneumococcus and meningococcus
212
What is the most appropriate antibiotic regime for a child over 3 months with suspected Meningitis?
IV ceftriaxone - 80 mg/kg once daily dose
213
What is the most appropriate antibiotic regime for a child under 3 months with suspected Meningitis?
IV Cefotaxime + Either Amoxicillin or Ampicillin to cover Listeria
214
Give a contra-indication to the prescription of Ceftriaxone
Concurrent calcium infusion
215
If a patient is receiving a calcium infusion, which antibiotic should be given in place of Ceftriaxone?
Cefotaxime
216
Give 3 bacterial pathogens that commonly cause Meningitis in neonates up to 3 months of age
1. Group B streptococcus
2. E.Coli
3. Listeria Monocytogenes
217
Give 3 bacterial pathogens that commonly cause meningitis in children between 3 months and 5 years of age
1. Niesseria Meningitides
2. Streptococcus pneumoniae
3. Haemophilus influenza B
218
Viral Meningitis in children is most commonly caused by which pathogens?
~ 85% are caused by enteroviruses such as Coxsackie B virus and echovirus
219
Give 6 risk factors for bacterial meningitis in childhood
1. Low family income
2. Asplenia
3. Maternal infection and pyrexia at the time of delivery
4. Children with facial cellulitis, periorbital cellulitis, sinusitis and/or septic arthritis
5. Basal skull fracture
6. Attendance at day care/ crowding
220
Recall 4 indications for dexamethasone adjunct therapy in the treatment of suspected/ confirmed meningitis
1. Frankly purulent pus
2. CSF WCC >1000
3. Raised CSF WCC with CSF protein >1g/l
4. Presense of bacteria on gram staining
221
Name the 2 principle pr-inflammatory markers that are implicated in the inflammatory response associated with sepsis
1. TNF- alpha
| 2. IL-1
222
Why are children with sickle cell disease at an increased risk of developing sepsis?
These patients will have functional asplenia due to sequestering of sickle cells within the spleen and subsequent fibrosis
223
Suggest 7 potential contraindications to performing an LP on a child
1. Child is too unstable
2. Symptoms/signs of raised intracranial pressure
3. Suspected intracranial mass e.g. brain tumour
4. After convulsions until the patient has stabilised
5. Extensive or spreading purpuric rash
6. Bleeding disorders
7. Local infections at LP site
224
Outline the course of antibiotics prescribed to a child who is under 3 months and has group B strep Meningitis
Minimum 14 days of ceftriaxone
225
Outline the course of antibiotics prescribed to a child who is under 3 months and has Listeria Meningitis
Minimum 21 days of amoxicillin plus gentamicin for at least the first 7 days
226
Outline the course of antibiotics prescribed to a child who is under 3 months and has a gram negative Meningitis
Minimum of 21 days of cefotaxime
227
Outline the course of antibiotics prescribed to a child who is over 3 months and has H influenza B meningitis
10 days of ceftriaxone
228
Outline the course of antibiotics prescribed to a child who is over 3 months and has Strep pneumoniae meningitis
14 days of ceftriaxone
229
Outline the course of antibiotics prescribed to a child who is over 3 months and has meningococcal sepsis
7 days of ceftriaxone
230
Give 5 potential acute complications associated with meningitis
1. Seizures
2. Raised intracranial pressure
3. Metabolic disturbance
4. Coagulopathy
5. Anemia
231
Give 5 potential long term complications associated with meningitis
1. Hearing problems
2. Psychosocial problems
3. Epilepsy
4. Developmental/ learning difficulties
5. Neurological impairment
232
Which common causative pathogen of meningitis in childhood has the highest mortality rate?
Streptococcus pneumoniae - these patients are also at the highest risk of neurological complications
233
What is the causative agent of 'slapped cheek syndrome'?
Parvovirus
234
A young child being discharged from hospital following treatment for bacterial meningitis should receive a follow up audiology assessment within how long?
Within 2 weeks of discharge
235
Name 3 groups of accessory muscles that are utilised when a child is in respiratory distress
1. Sternocleidomastoid
2. Parasternal
3. Scalene muscles
236
What is the physiological definition of pulsus paradoxus?
A fall in systolic BP >10 mmHg on inspiration
237
In an infant under 3 months, what is the significance of a fever > 38 degrees?
Indicative of likely bacterial meningitis/ sepsis
238
What is the most common cause of proteinuria?
Glomerular disease accounts for ~ 90% of cases seen
239
How is proteinuria diagnosed clinically?
> 20 mg/mmol Protein:creatine ratio
240
A Protein:creatine ratio of what level would be expected in a 'nephrotic presentation'
Very high Ratio i.e. >200mg/mmol
241
Which biochemical value can help distinguish between glomerular and tubular proteinuria?
An albumin:creatine ratio
242
Recall the clinical triad associated with nephrotic syndrome
1. Massive proteinuria
2. Hypoalbuminaemia - <25g/l
3. Oedema
243
Give 3 'thrombotic ' complications associated with nephrotic syndrome
1. Haemoconcentration
2. Increased fibrinogen, factor VII, VIII and X
3. Decreased antithrombin III and plasminogen
244
Recall 2 infections that are recognised as potential complications of nephrotic syndrome
1. Pneumococcal infections
| 2. Primary peritonitis
245
Give 3 complications of minimal change nephrotic syndrome in childhood
1. Spontaneous peritonitis
2. Streptococcal infection
3. Recurrence of disease
246
What are the 3 main histological types of congenital kidney malformation?
1. Renal hypoplasia - fewer nephrons than normal
2. Renal dysplasia - undifferentiated kidneys +/- cysts
3. Renal agenesis
247
Mutation in which gene is linked to 'renal cyst and diabetes syndrome'
HNF1B
248
Mutation in which gene is linked to renal coloboma syndrome?
PAX2
249
Mutation in which gene is linked to Ochoa or Uro-facial syndrome?
HPSE2
250
What is the inheritance pattern of congenital renal agenesis?
Autosomal dominant with incomplete penetrance
251
What is the inheritance pattern of Potter's syndrome?
Autosomal recessive
252
What is Ask-Upmark kidney?
Segmental renal hypoplasia - associated with severe juvenile hypotension
253
Recall 4 extra-renal components of APKD
1. Hepatic and pancreatic cysts
2. Chronic hypertension
3. Intracranial aneurysms
4. Cardiac valve anomalies - particularly mitral valve prolapse
254
Suggest 5 complications associated with a horseshoe kidney
1. Stone disease
2. PUJ obstruction
3. Trauma
4. Infection
5. Tumours
255
What is the pathophysiology of medullary sponge kidney?
Cystic dilatation of the collecting tubules in one or both kidneys
256
What is the pathophysiology of Ureterocele?
Sacculation of the bladder end of the ureter - this can occur either in the bladder or ectopically
257
Recall the classic triad associated with Prune-belly syndrome
1. Urinary tract anomalies
2. Deficient abdominal musculature
3. Bilateral cryptochidism
258
Define bladder exstrophy
Absence of the anterior wall of the bladder
259
Name 2 medical conditions that can result in hypogonadotropic hypogonadism
1. Kallmann's syndrome
| 2. Prader-Willi syndrome
260
What causes the formation of a varicocele?
Dilation of the pampiniform plexus (spermatic venus plexus)
261
At what point during gestation do the kidneys begin to form?
Week 5 - Glomeruli continue to form until 34 weeks gestation
262
What is the most common congenital renal abnormality?
Renal hypoplasia?
263
What is the most common causative organism of a UTI in childhood?
E.Coli
264
Give 3 diagnostic criteria which may indicate an upper urinary tract infection in a child
1. Bacteriuria and fever of 38 degrees or higher +/- loin tenderness
2. Bacteriuria, loin pain/ tenderness and fever of less than 38 degress
3. Age less than 3 months
265
A renal ultrasound must be arranged within what time frame for a child being discharged following treatment for a UTI
6 weeks
266
Which 3 investigations are used to to investigate a child with UTI?
1. Renal ultrasound
2. MCUG - Micturating cystogram
3. DMSA radionucleotide scan
267
What is the most common cause of hydronephrosis seen in young children?
Vesicoureteric reflux
268
If scarring of the kidney is found on DMSA scan. what is the % risk of developing hypertension in later life?
10%
269
Recall the 3 main physiological factors involved in nocturnal enuresis
1. Defective sleep arousal
2. Nocturnal polyuria
3. Bladder factors e.g. reduced bladder capacity
270
Suggest 3 potential pharmacological agents which may be used in the treatment of nocturnal enuresis
1. Desmopressin
2. Imipramine
3. Anticholinergic drugs
271
What is countercurrent multiplication with reference to the physiology of the kidney?
The use of an osmotic gradient in the loop of Henle to absorb water and thereby concentrate the urine
272
Give 5 clinical sequelae that would give clinical suspicion of acute kidney injury during the assessment of a child
1. Oedema
2. Poor urine output <0.5 mls/kg/hr for at least 8 hours
3. Increased serum creatine or urea
4. Decreased glomerular filtration rate
5. eGFR < 0.55mg/dl (ages 1 -13 years)
273
Bilateral dilated kidneys in a male infant may be indicative of what serious condition?
Posterior urethral valves - may lead to renal failure secondary to obstruction if left untreated
274
Ascites as a result of nephrotic syndrome leaves a patient at risk of which serious complication?
Pneumococcal sepsis
275
How is the potential risk of pneumococcal sepsis in a child with nephrotic syndrome managed?
Prophylactic penicillin
276
Name the only test that can reliably be used to identify the presence of vesoureteric reflux in a child
Micturating cystogram
277
What is Wiskott-Aldrich syndrome?
Rare X linked recessive condition characterised by:
- Eczema
- Thrombocytopenia
- Immune Deficiency
- Bloody diarrhoea (secondary to thrombocytopenia)
278
What is the most common form of vasculitis seen in childhood?
Henoch-Schonlein Purpura
279
Recall the classical tetrad associated with Henoch- Schonlein Purpura
1. Rash
2. Abdominal pain
3. Arthritis/ arthralgia
4. Glomerulonephritis
280
What are the 6 potential treatment options for the management of ITP in a child?
1. No treatment
2. Tranexamic acid
3. Steroid therapy
4. IV Immunoglobulin
5. Anti- D
6. Splenectomy
281
RB 1 Gene is associated with which type of childhood cancer?
Retinoblastoma
282
Give 4 tumour types which may lead to a child presenting with a head tilt
1. Retinoblastoma
2. Rhabdosarcoma
3. Medulloblastoma
4. Optic glioma
283
Visible unexplained haematuria could be a red flag symptom for what form of childhood cancer?
Wilm's tumour
284
Unexplained hepatosplenomegaly could be a red flag symptom for what form of childhood cancer?
Leukaemia
285
Breathlessness in addition to generalised lymphadenopathy and splenomegaly could be a red flag symptom for what form of childhood cancer?
Lymphoma
286
An absent red reflex could be a red flag symptom for what form of childhood cancer?
Retinoblastoma
287
A 6th nerve palsy would cause paralysis of which eye muscle - observable during a H test assessment
Paralysis of the lateral rectus muscle - i.e. patient would be unable to follow examiners finger laterally in the affected eye
288
What is the inheritance pattern of neruofibromatosis?
Autosomal dominant
289
What is the most common cause of iron deficiency anemia seen in young children in the UK?
Insufficient dietary intake
290
Name 2 antibiotics that can be used in the treatment of Lyme disease
1. Cefuroxime
| 2. Amoxicillin
291
What type of infection can be caught through contact with lizards? (Yes this is a serious question)
Salmonella which can be transferred to humans
292
Define Kernig's sign
Pain on lower leg extension with the hip flexed
293
Define Brudzinski's sign
Involuntary flexion of the hips and knees upon neck flexion
294
What is the most common cause of infective diarrhoea in children seen in the UK?
Rotavirus
295
What type of rash is commonly seen in Lyme disease?
Erythema migrans
296
What is the first line medical therapy for the treatment of Kawasaki disease? (2)
1. IV gamma globulin
| 2. Oral aspirin
297
According to the NHS vaccination schedule, at the age of 8 weeks a child should receive which vaccinations?
1. 6 in 1 vaccine
2. Rotavirus
3. MenB
298
According to the NHS vaccination schedule, at the age of 12 weeks a child should receive which vaccinations?
1. 6 in 1 vaccine (second dose)
2. Pneumococcal vaccine
3. Rotavirus (second dose)
299
According to the NHS vaccination schedule, at the age of 16 weeks a child should receive which vaccinations?
1. 6 in 1 vaccine (third dose)
| 2. MenB (second dose)
300
According to the NHS vaccination schedule, at the age of 1 year a child should receive which vaccinations? (4)
1. Hib/Men C (first booster)
2. MMR (first dose)
3. Pneumococcal (second dose)
4. Men B (third dose)
301
According to the NHS vaccination schedule, at the age of 3 years and 4 months a child should receive which vaccinations?
1. MMR (second dose)
| 2. 4 in 1 preschool booster
302
According to the NHS vaccination schedule, at the age of 12 to 13 years a child should receive which vaccinations?
HPV vaccine
303
According to the NHS vaccination schedule, at the age of 14 a child should receive which vaccinations?
1. 3 in 1 teenage booster
| 2. Men ACWY
304
During the management of a child with status epilepticus, what tis the fist medication to be administered?
If vascular access is available - Lorazepam 0.1 mg/kg
| If no vascular access - Buccal midazolam 0.5 mg/kg or rectal diazepam 0.5 mg/kg
305
What are the 9 main differentials to consider when presenting with a child with a reduced level of consciousness?
1. Shock
2. Sepsis
3. Trauma
4. Metabolic illness
5. Intracranial infections
6. Raised ICP
7. Hypertension
8. Prolonged convulsions
9. Post-convulsive state
306
What is the most common type of seizure disorder seen in childhood?
Febrile seizures
307
Define a simple febrile seizure
Short generalised seizure with a duration of under 15 minutes. The seizure does not reoccur within 24 hrs and is seen during a febrile episode not caused by an acute disease of the nervous system in a child aged 6 months to 5 years
308
What are the 2 main differential diagnoses for a febrile seizure?
1. Syncope during a febrile state
| 2. Abnormal motor manifestations
309
A patient can be diagnosed with epilepsy if any one of which 3 criteria are met?
1. At least 2 unprovoked seizures occurring > 24hrs apart
2. One unprovoked seizure and the probability of further seizures similar to the general recurrence risk (at least 60%) after 2 unprovoked seizures, over the next 10 years
3. Diagnosis of an epilepsy syndrome
310
What is Sturge-Weber Syndrome?
Over abundance of capillaries seen in the meninges and in the areas of the face innervated by the trigeminal nerve - leads to characteristic 'port wine stain' birth marks on the faces of infants born with the condition
311
What are the 2 main groups of inborn errors of metabolism which may cause hyperammonaemia?
1. Urea cycle disorders
| 2. Organic acidaemias e.g. Methylmalonic or propionic acidemia
312
What is the normal inheritance pattern for an inborn error of metabolism?
Autosomal recessive
313
With regards to Group B streptococcus infections in neonates, define early and late onset infection
Early onset - occurs within the first 6 days of life
| Late onset - from 7 days to 3 months after birth
314
Give 5 cohorts of women who would be offered antibiotic therapy during labour to protect against neonatal Group B streptococcus infection
1. Previous Hx of Group B strep infections in previous births
2. Group B strep cultured from the mothers urine at some stage during pregnancy
3. Fever during labour
4. Labour before 37th week of pregnancy
5. Waters break early
315
How does the classification of an inborn error of metabolism help to predict the clinical sequelae of the disease state?
Small molecule diseases - often episodes of acute encephalopathy
Organelle disease - Typically seen as chronic neurodegeneration
316
Give 5 modes of clinical presentation for an inborn error of metabolism
1. Screening process
2. Dysmorphism
3. Acute encephalopathy
4. Liver disease
5. Chronic neurodegeneration
317
Recall the 2 main clinical characteristics of Phenyketonuria
1. Severe developmental delay and/or regression of developmental milestones
2. Seizures
318
What is Zellweger syndrome?
An absence of peroxisomes leads to structural abnormalities in the brain, kidneys and bones. Seizures are common and the infant is often extremely floppy on examination
319
What is Hurler disease?
A form of mucopolysaccharidosis characterised by progressive restructuring. Features of the condition include:
- Coarse facial features
- Hepato and splenomegaly
- Skeletal abnormalities
Condition brought about by a missing lysomal enzyme
320
Recall the 3 main types of biochemical abnormality that may give rise to acute encephalopathy
1. Hypoglycaemia
2. Metabolic acidosis
3. Hyperammonaemia
321
Leigh syndrome is an example of what kind of inborn error of metabolism?
Mitochondrial disorder
322
What is the most common inborn error of metabolism associated with the urea cycle?
Ornithine transcarbamylase deficiency - X linked condition
323
What are the 2 most common identified causes of cognitive impairment in infants?
1. Down's syndrome
| 2. Foetal alcohol syndrome
324
What percentage of people with autism spectrum disorder also suffer from epilepsy?
~ 20-30%
325
What are the 3 main causes of profound disability?
1. Chromosomal abnormalities
2. Cerebral palsy
3. Inborn errors of metabolism
326
What is the definition of cerebral palsy?
A disorder of tone, posture and movement, caused by a non progressive brain lesion in a developing brain
327
Name 3 different types of cerebral palsy
1. Spastic
2. Ataxic
3. Dyskinetic
328
Recall 3 medications that can be used to help manage increased tone in children with cerebral palsy
1. Baclofen
2. Diazepam
3. Botulinum toxin
329
Autoantibodies produced in idiopathic thrombocytopenic purpura are most commonly targeted towards which surface glycoproteins?
Platelet surface glycoproteins - most commonly IIb and II a
330
Thrombocytopenia in a patient with Down's syndrome may indicate the development of which condition and requires urgent follow up?
Megakaryoblastic leukaemia
331
Recall the 5 developmental domains
1. Gross motor
2. Fine motor
3. Cognition
4. Speech and language
5. Personal/ social
332
What are the 3 principle characteristics of HELLP syndrome?
1. Haemolysis
2. Elevated Liver enzymes
3. Low platelets
333
What are the 2 most common types of leukaemia found in children?
1. Acute lymphoblastic leukaemia
| 2. Acute Myeloid leukaemia
334
Give 3 genetic conditions that greatly increase a child's risk of developing leukaemia
1. Down's syndrome
2. Neurofibromatosis
3. Schwachman syndrome
335
The disease progression of Duchenne muscular dystrophy can be slowed with which medication?
Steroids
336
What plasma glucose level in a child/ young person warrant clinical suspicion of DKA in a patient who is not a known diabetic?
> 11 mmol/L
337
What ketone serum level is sufficient to diagnose Ketonaemia?
Blood beta-hydroxybutyrate > 3mmol/L
338
Recall the assumed fluid deficits used for the calculation of the fluid requirements of a child with DKA
- 5% fluid deficit for children with mild to moderate DKA ( blood pH > 7.1)
- 10 % deficit for children with severe DKA
339
What are the 'reduced volume' rules used to calculate maintenance fluid requirements for children with DKA?
- < 10kg , give 2 mls/kg/hr
- Between 10-40kg 1 ml/kg/hr
- > 40kg , give a fixed volume of 40 ml/hr
340
Why are lower fluid maintenance levels used in the management of children with DKA?
To help reduce the risk of cerebral oedema
341
Give 4 clinical signs which may be early manifestations of cerebral oedema in a child presenting with DKA
1. Headache
2. Agitation
3. Irritability
4. Unexpected fall in heart rate/ increase in BP
342
What are the 2 options for the immediate treatment of cerebral oedema in a child?
1. Mannitol 20% 0.5-1 g/kg over 10-15 minutes
| 2. Hypertonic sodium chloride 2.7% or 3% 2.5-5 mls/kg over 10-15 minutes
343
A positive GAD test in a child can be indicative of which medical condition?
Type 1 diabetes
344
What are the optimal targets for blood glucose control? (4)
1. Fasting plasma glucose level 4-7 mmol/L on waking
2. Plasma glucose level 4-7 mmol/L before meals/ at other times of the day
3. Plasma glucose 5-9 mmol/L after a meal
4. Plasma glucose of at least 5 mmol/L when driving
345
What doses of intramuscular glucagon can be administered to children for the treatment of severe hypoglycaemia in the hospital setting?
- Children > 8 yrs ( or weigh > 25 kg) 1mg of glucagon
| - Children < 8 yrs )or those < 25 kg) 0.5 mg glucagon
346
Give 4 conditions that children with type 1 diabetes require regular monitoring for
1. Thyroid disease annually
2. Diabetic retinopathy - annually from 12 yrs
3. Increasing albuminuria - can indicate diabetic kidney disease
4. Hypertension - annually from 12 yrs
347
Give 3 rare complications associated with type 1 diabetes in children
1. Juvenile cataracts
2. Necrobiosis lipoidica
3. Addison's disease
348
Give 2 conditions that have a recognised increased maternal incidence with cases of foetal fatty acid oxidation disorder
1. AFLP - Acute fatty liver of pregnancy
| 2. HELLP syndrome
349
What is a consequence of foetal steroid sulphatase deficiency on pregnancy?
Prolonged labour - due to decreased placental oestrogen production
350
What is the classical description of a venous hum (form of benign childhood murmur)?
Continuous blowing noise heard below both clavicles - only heard in children
351
What is the SSRI of choice in children and adolescence?
Fluoxetine
352
What is the appropriate management of a child with ? Eczema Herpeticum?
Urgent referral to hospital for confirmed diagnose - medical emergency that would require IV antiviral therapy
353
What is the most common causative organism of bronchiolitis?
Respiratory syncytial virus
354
Recall the additional sections to include in the Paediatric history? (mnemonic BINDS)
```
B - Birth
I - Immunisation
N - Nutrition/feeding
D - Development
S - Social Hx in detail; including school, siblings, smoking (parents), salaries(parents jobs) and social services input
```
355
Recall the systematic review questions to ask in paediatrics (WAFFLESS)
```
W - Weight
A - Appetite
F - Fever
F -Feeding
L - Lumps
E - Energy/mood/crying
S - School absences
S -Sleep disturbance
```
356
A history of intussusception is a contra-indication for which childhood vaccination?
Rotavirus vaccine - as the vaccine is thought to increase the risk of reoccurrence
357
Paediatric heel prick screening test checks for which 9 diseases? (C H2 A2 M2 P S)
```
C - Cystic fibrosis
H - Hypothyroidism
H - Homocystinuria
A - Acidemia (glutaric)
A - Acidemia (isovaleric)
M - MCADD
M - Maple syrup urine disease
P - PKU
S - Sickle cell
```
358
Briefly define Rett syndrome
Neurodevelopemental disorder most commonly see in girls. Characterised by slowing down of development and eventual global impairment leading to learning disability
359
Hand preference before 18 months may indicate what? (2)
1. Hemiplegia
| 2. Brachial plexus injury
360
Suggest 5 potential causes of delay in gross motor development
1. Cerebral Palsy
2. Dysplasia of the hip
3. Spina Bifida
4. Duchenne muscular dystrophy + other neuromuscular disorders
5. Achondroplasia
361
Define kernicterus
A complication of neonatal jaundice whereby an elevated level of bilirubin in the blood leads to encephalopathy. Predominantly seen in pre-term infants.
362
What is the mutation associated with DMD?
X-linked mutation of dystrophin
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Cystic fibrosis is caused by a mutation on which chromosome?
Chromosome 7
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What is the most common causative organism of croup?
Para influenza virus 1-4 (accounts for ~ 80% of cases)
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What is the surgical treatment for pyloric stenosis?
Ramstendt Pyloromyotomy
| - involves dividing the pyloric muscle to increase the diameter of the gastric outlet
