Child Health Flashcards
(299 cards)
1
Q
Why should you not examine the upper airway in suspected airway obstruction?
A
As it can lead to increased distress and breathlessness
2
Q
In which respiratory infections should you not examine the upper airway and why?
A
Croup
Epiglottitis
As it can cause further obstruction
3
Q
In what age group is croup most common?
A
6 months - 3 years
Peaks at age of 2
4
Q
What is the main cause of croup?
A
Viral respiratory infection - typically parainfluenza
Causes infection of the upper airways
5
Q
What are the differentials for upper airway obstruction in children?
A
Epiglottitis Foreign body aspiration Croup Allergic reaction Tonsillar abscess
6
Q
How can you tell the difference between mild, moderate and severe croup?
A
Mild - just seal like barking cough
Moderate - plus stridor and sternal recession
Severe - plus agitation, lethargy, decreased level of consciousness. Signs of respiratory failure
7
Q
What is the management of croup?
A
Mild - oral dexamethasone
Moderate - plus nebulised adrenaline
Severe - hospital admission, oxygen, nebulised adrenaline and dexamethasone
8
Q
What is epiglottitis?
A
Inflammation and swelling of epiglottis
9
Q
What are the symptoms of epiglottitis
A
Dyspnoea Dysphonia (difficulty speaking) Dysphagia (difficulty swallowing) Fever Irritability Pooling of oral secretions and saliva
10
Q
How is epiglottitis managed?
A
Hospital admission
Intubation
IV antibiotics
Corticosteroids
11
Q
Which bronchus are foreign body aspiration more likely to go down and why?
A
Right main bronchus
As the left one has a more acute angle
12
Q
Why is inhalation of a circuit battery dangerous?
A
Can set up an electrical circuit in moist environment
This can cause oesophageal perforation
13
Q
What is laryngomalacia?
A
Congenital abnormality of the larynx cartilage
This predisposes a child to dynamic supraglottic collapse during inspiratory phase of respiration
14
Q
When does laryngomalacia present?
A
In first few weeks of life
Usually spontaneous resolution by 2 years
15
Q
Why is the presentation of epiglottitis in children becoming more uncommon?
A
Due to the HiB vaccination
16
Q
What are the actions of histamine?
A
Vasodilation
Bronchoconstriction
Localised irritation
Endothelial cell separation
17
Q
When would you prescribe someone an adrenaline pen?
A
If they have a history of anaphylaxis - with previous cardiovascular and respiratory involvement
Evidence of previous airway obstruction
Poorly controlled asthma
If they react to small amounts of allergen
If they cannot easily avoid an allergen
18
Q
What is the feeding requirements for neonates?
A
150ml/kg/day - up to 1 month
100ml/kg/day - after 1 month
19
Q
In which congenital heart conditions would the lung fields appear brighter on a chest X-ray?
Why is this?
A
Ventricular septal defect
Patent ductus arteriosis
Cardiac failure
This is because too much blood is going to the lungs
20
Q
In which congenital heart conditions would the lung fields appear darker on a chest X-ray?
Why is this?
A
Fallouts tetralogy
Pulmonary stenosis
Too little blood going to the lungs
21
Q
What are the risk factors for a neonate having congenital heart disease?
A
Rubella during pregnancy
Diabetes during pregnancy
Using ACEi, statins, lithium during pregnancy
Smoking/drinking during pregnancy
Consanguinity - where parents are blood relatives
22
Q
What are the different aycanotic congenital heart defects?
A
Ventricular septal defect Arterial septal defect Patent ductus arteriosis (PDA) Coarction of the aorta Aortic valve stenosis
23
Q
What are the different cyanotic congenital heart defects?
A
Tetralogy of fallout (TOF)
Transposition of the great arteries (TGA)
Ebsteins anomaly
24
Q
At what oxygen saturations would cyanosis start to occur?
A
Between 80-85% of oxygen
25
What is the most common congenital heart disease?
Ventricular septal defect (VSD)
26
What kind of murmur is heard in VSD?
Pan systolic murmur
27
How does VSD present in neonates?
Usually asymptomatic in first few weeks of life
After that they present with poor feeding, often becoming breathless when feeding, and poor weight gain
28
How is VSD diagnosed?
Echocardiogram
29
What causes the PDA close after birth
High circulating oxygen
| Fall in prostaglandin
30
Why might a PDA stay open after birth?
Preterms - as lungs are less mature so less sensitive to oxygen
Sensitivity to low levels of prostaglandin - so stays open
Rubella infection
31
What are the signs of a PDA?
Continuous machinery murmur - left scapula
Large volume, bounding, collapsing pulse
Wide pulse pressure
Left subclavicular thrill
32
How is a PDA managed?
Indomethacin (NSAID) - which inhibits prostaglandin synthesis and causes PDA to close
33
What conditions are screened for at the neonatal heel prick test?
Sickle cell disease
Cystic fibrosis
Congenital hypothyroidism
Inherited metabolic diseases (x6)
34
Why might you not give high flow oxygen to a baby with heart failure?
If you were relying on the PDA to maintain a viable circulation
High oxygen concentrations can cause duct failure and lead to worsening of condition
35
What is the pathophysiology of cystic fibrosis?
Mutation in chloride transporter
Chloride cannot be pumped out of cells, which is usually responsible for dragging water out with it
This results in not enough water being dragged into the airways
This results in the mucus being thick and sticky, blocking the airway
Mucus is usually responsible for trapping bacteria and being wafted out by cilia
If Mucus is thick it is unable to be cleared, which can result in difficulty breathing and increase risk of respiratory infections
36
How might cystic fibrosis be diagnosed?
Neonatal heel prick test
Sweat test - check for chloride levels
Genetic test
X ray - may show signs of bronchiectasis
Stool analysis - as high levels of nutrients may suggest poor absorption
37
What chemical is used during the sweat test?
Pilocarpine - applied to skin which causes sweating
38
How does cystic fibrosis in children commonly present?
Neonatally - can present with meconium ileus, prolonged jaundice
In childhood - may present as recurrent chest infections, steatorrhoea, or failure to thrive
39
How is cystic fibrosis managed?
Physiotherapy - to clear airways
Antibiotics - to treat recurrent chest infection
Medicines to make mucus in lungs thinner - e/g, mannitol dry powder
Bronchodilators - to widen airways and make breathing easier
Pancreatic enzyme supplements
40
What are the main causes of bronchiectasis in children?
```
Cystic fibrosis
Primary ciliary dyskinesia
Severe respiratory tract infection
Immune deficiency
Poor lung development
Blockage due to nut
```
41
What is appropriate diet for a child with cystic fibrosis?
High calorie
High fat
Pancreatic enzyme supplementation for every meal
42
What is the stepwise management for a child aged 5-16 with asthma?
1st line - SABA
2nd line - low dose ICS
3rd line - leukotrine receptor antagonist (LTRA)
4th line - low dose ICS and LABA (stop LTRA)
5th line - maintenance reliever therapy (MART) with ICS
43
What is Harrison's sulcus?
It is a horrizontal groove on the lower border of the thorax corresponding to the costal insertion of the diaphragm
It is an indicator of chronic asthma in children or obstructive respiratory disease
44
What is the presentation of asthma in a child?
```
Recurrent viral respiratory infections
Wheeze
Dry cough
Breathlessness
Symptoms typically worse at night
```
45
Any safeguarding concerns should be reported to who?
Social services
Also report to the safeguarding lead - this may be a designated doctor or nurse
46
What is the pathophysiology of shaken baby syndrome?
Babies have weak neck muscles
Therefore if an infant is shaken their head will move uncontrollably
This movement causes the baby's brain to be thrown against side of skull - which can cause bruising, swelling or bleeding
47
What are the 3 conditions that may indicate shaken baby syndrome?
Encephalopathy
Subdural haematoma
Retinal haemorrhage
48
What imaging would you do if you suspected shaken baby syndrome?
MRI/CT scan - look for any bleeding or swelling in brain
Skeletal x ray - may reveal fractures
Ophthalmic exam - looking for retinal haemorrhage
49
What are the differentials for bruising in a child?
```
Accidental injury
Non accidental injury
Clotting disorder - Von willbrands, haemophilia, thrombocytopenia
Henoch-scholein purpura
Meningococcal septicaemia
Leukamia
Mongolian blue spot
```
50
What investigations would you do if a child presented with bruising?
Bloods - FBC, U&E, LFTs, clotting screen, bone profile
Blood film
INR and APTT - if appropriate
51
What are the differentials for Fracture in a child?
```
Non accidental injury
Accidental injury
Brittle bone disease (osteogenesis imperfecta)
Copper deficiency
Vit D deficiency
Vit C deficiency
Ehlers Danlos syndrome
JOBs syndrome
```
52
What are the differentials for burns/scalds in a child?
Non accidental injury - especially if glove stocking syndrome
Accidental injury
Bulbous impetigo - bacterial infection
Staphylococcal Scalded skin syndrome (SSSS)
53
What are the signs of a fabricated or induced illness (FII)?
Often on background of existing disease
OR bizzare new illness
Strange new symptoms
Parental reportage not keeping with physical findings
Symptoms not witnessed by other (e.g, fits not seen by school)
54
What is a child protection medical assessment?I
Medical assessment requested by social services
Carried out by a consultant
Detailed history and exam taken which is recorded
Any other relevant investigations done
The doctor will then produce a report for social services about the likelihood of an injury being accidental or not
55
What are the red flags for abuse regarding bruising in children?
If on face, back or buttock
If bruise outlines an object e/g, hand, belt
If there is fingertip bruising
56
What are the toxic trio for child abuse?
1) domestic violence
2) parental mental health problem
3) parental substance abuse problem
57
What is a body map and when is it used?
It is used to accurately document visible findings on examination
58
What are the differentials for chronic abdominal pain in children?
```
Chronic recurrent abdominal pain syndrome
Constipation
Peptic ulcer disease
IBS
IBD
Abdominal migraine
```
59
Which type of IBD is more common in children?
Crohn's disease
60
How is Crohn's disease managed in children?
Diet - liquid protein diet is successful in achieving remission
Then slowly introduce different foods to see what is safe
Medication - steroids, immunosuppressive meds, biologics - these can be used if diet is unsuccessful
61
When should you diagnose a functional gut disorder in a child with recurrent abdominal pain?
When all other disorders have been ruled out
62
What are the causes for blood in stools in children?
Bacterial infection - e.g campylobacter, salmonella
IBD
Tearing from anal vein
Intussusception - note that child would present acutely unwell
63
What are the differentials for acute abdominal pain in a child?
```
Gastroenteritis
DKA
Appendicitis
Intussusception
UTI
Henoch-sholein purpura
Testicular torsion
```
64
At what age is intussception most common?
Between the ages of 3 months - 2 years
65
What are the causes of intussusception?
May have preceding viral illness
Swelling in the bowel wall
Meckels diverticulum (congenital outpouching)
66
What are the symptoms of intussusception?
Severe abdominal pain
Child may get pain when they draw the legs up
Dehydration signs - fewer wet nappies, sunken fontanelle, dry mucous membranes
Fever
Acitis
Blood in stool - red currant jelly stool
67
How would you diagnose intussusception?
Abdominal exam - can sometime palpate it
| USS - doughnut sign
68
How is intussusception managed?
Fluids - rehydrate child
Nasogastric tube - to drain stomach and bowel contents
Antibiotic - to reduce infection
Air enema - passed up child bottom and releases air. This has effect of pushing the bowel back
69
What should you always ask any child that presents with diarrhoea and vomiting?
Travel history
Contact history - anybody else ill, any animals?
Vaccination history
70
Which conditions can cause weight loss in children?
```
Coeliac disease
Type 1 diabetes
Hyperthyroidism
IBD
Malignancy
```
71
Which mental health problems may cause weight loss in children?
Anorexia nervosa
Depression
OCD
Autism
72
What are the risks of rapid weight loss?
How can you check for these?
Hypoglycaemia - check BMs
Risk of infection - FBC
Cardiac arrhythmia - ECG
73
What are the 3 main eating disorders seen in young people?
Anorexia Nervosa
Bulimia Nervosa
EDNOS
74
What are the behavioural features of anorexia nervosa?
Feeling fat, unhappy with body shape
Won't eat in front of others
Hiding food
Compulsive exercise
75
What are the clinical features seen in someone with anorexia nervosa?
```
Weight loss
Amenorrhoea
Headaches - due to dehydration
Constipation - as gastric motility slows down
Dry skin and hair loss
Lethargy
Dizziness/fainting
Cool peripheries
```
76
What are the features of bulimia nervosa?
Binging on food and then vomiting
Can occur alongside self harm
Weight is often normal
Associated with low self worth
77
Why do people with eat disorders abuse laxatives?
They struggle with sensation of food in their bodies
| So take laxatives to relieve this feeling
78
What are the side effects of laxatives?
Electrolyte imbalances - can lead to cardiac arrest
Dehydration
Oedema - happens when patients stop taking them, which leads to fluid overload
Loss of bowel motility - in chronic laxative use
79
What is the cause of anorexia nervosa?
Neuro-biological illness - important to explain that it is not the patients fault
80
What does emancipated mean?
When a patient looks abnormally thin
81
What is Lanugo hair?
Hair on face that happens in anorexia nervosa - this is a response to the loss of the insulating effect of fat tissue
82
Why is weight for height ratio used instead of BMI in children?
BMI is less reliable in children
Can assess the height to their age
83
At what ratio would the weight to height ratio concern you?
If less than 75%
84
How would you manage a patient with anorexia nervosa?
Hospital admission for stabilisation
Encourage to eat - aim for 0.5-1kg weight gain per week
Commence on vitamins (thiamine, vit B, multivitamins)
When stable can discharge and support with multiple teams in community - child psychiatrist, dietician, family therapist, paediatrician
85
What bloods do you have to monitor when treating a patient with anorexia nervosa and why?
Must monitor phosphate
Drop in phosphate is a marker of re feeding syndrome
This may precipitate respiratory arrest
86
What are the differentials for vomiting in a child?
```
Feeding issues
Gastroesophageal reflux disease
Gastroenteritis
Pyloric stenosis
Meningitis
Urinary tract infection
Coeliac disease
Intussusception
```
87
What are the signs of dehydration in a child?
```
Sunken anterior fontanelle
Dry mucous membranes
Tachycardia
Oliguria (lack of urination)
Reduced capillary refill time (>3 seconds)
Reduced skin turgor
```
88
Which children are at an increased risk of dehydration?
Low birth weight
Age <1 year
More than 2 vomiting episodes and more than 5 diarrhoea episodes in last 24 hours
89
How would you manage dehydration associated with vomiting/diarrhoea in children?
Rehydration with oral rehydration solution
Give by nasogastric tube if child is unable to drink or there is persistent vomiting
Give fluids in severe dehydration
Also remember to monitor urine output, as well as the number of vomit and stool
90
How do oral rehydration solutions (ORS) work?
They provide a mix of glucose, electrolytes and salts, which help with the absorption of sodium in the intestines
As the sodium is absorbed, then water is able to follow - helping the body to rehydrate
91
What are the signs of hypernatraemia?
```
Jittery movements
Increased muscle tone
Hyperreflexia
Convulsions
Drowsiness or coma
```
92
What are the main causes of gastroenteritis in children?
Rotavirus - especially in children under the age of 2
Adenovirus - more common in young children
Norovirus - more common in adults
Campylobacter
C.diff
Salmonella
Shigella
E.Coli 0157 - less common in children but should always be considered
93
When would you want to do further investigations in a child presenting with gastroenteritis?
```
Recent travel abroad
If diarrhoea isn't improving by day 7
In suspected septicaemia
If there is blood or mucus in stool
In an Immunocompromised child
```
94
What is the usual time course of gastroenteritis?
Vomiting should settle in 2-3 days
Diarrhoea should settle by day 5-7
Most children should recover completely within 2 weeks
95
How does haemolytic uraemic syndrome present?
Bloody diarrhoea
Abdominal pain
Nausea and vomiting
96
What is the pathophysiology of HUS?
E.coli 0157 produces shiga toxin
This binds to cells causing microvascular thrombosis, fragmentation of erthrocytes and destruction of platelets
This results in renal insufficiency
Also effects the hear, intestines, pancreas and CNS
97
How is HUS managed?
DO NOT USE ANTIBIOTICS - these can cause further renal damage
Mainly supportive treatment
Make sure patient is adequately hydrated - but take care to avoid fluid overload
Treat any anaemia with transfusion or dialysis
98
What fluids would you give in fluid resuscitation in a child?
20ml/kg 0.9% sodium chloride by rapid IV infusion
99
What fluids do you give in fluid maintenance for a child?
```
0.9% NaCl and 5% dextrose
A: 100ml/kg for first 0-10kg
B: 50ml/kg for 10-20kg
C: 20ml/kg for >20kg
(A+B+C=D)
To get the amount in ml/hour, do D divided by 24 hours
```
100
What are the main differentials for fever in child?
```
URTI
Meningitis
UTI
Bronchiolitis - if in 1st year of live
Kawasakis disease - if lasting longer than 5 days
Tonsillitis
Otitis media
Epiglottitis
Septic arthritis
```
101
How does Kawasaki's disease present?
```
Fever lasting more than 5 days
Cervical lymphadenopathy
Conjunctival infection (red eyes)
Cracked lips, strawberry tongue
Polymorphous rash - rash due to sensitively to sun
```
102
What group of patients would you be more likely to see kawasakis disease in?
Children under 5 years
Children from northeast Asia, especially japan and Korea
103
How would you manage a patient with suspected kawasakis disease?
IV immunoglobulin and aspirin - these reduce risk of coronary artery aneurysm
Steroids
ECHO - to check for coronary artery aneurysm
104
What are the differentials for a non-blanching rash in a child?
Meningitis
Henoch-scholein purpura
Idiopathic thrombocytopenia Purpura
Measles - rash can become non-blanching after 3-4 days
105
What is henoch-schonlein purpura?
Type of vasculitis that commonly occurs in children
Typically seen in children following an infection
Degree of overlap with IgA nephropathy (Berger's disease)
106
How does henoch-schonlein purpura present?
Palpable purpuric rash over buttocks and extensor surfaces and arms and legs
Abdominal pain
Polyarthritis (joint pain)
Features of IgA nephropathy e.g, haematuria, renal failure
107
How is HSP usually managed?
Supportive care as generally self limiting condition
Pain relief for analgesia
Check U&Es and PCR for kidney function
108
What are the non-specific symptoms that children can present with when they have meningitis?
```
Fever
Vomiting/nausea
Lethargy
Irritability
Refusing food/drink
URTI symptoms
```
109
What are the specific meningitis symptoms?
```
Stiff neck
Headache
Altered mental stat
Bulging fontanelle (in children <2)
Photophobia
Seizures
```
110
What are the kernigs and brudzinski's signs?
Signs that might suggest meningitis
Kernigs - pain in neck when hips are flexed to 90 degrees and knees extended
Brudzinski's sign - when neck is flexed this causes hips and knees to flex
111
What are the contraindications for doing a lumbar puncture?
```
Evidence of raised ICP
Focal neurological signs
Signs of shock
Signs of meningococcal septicaemia (Purpuric rash)
Coagulation abnormalities
Infection at puncture site
```
112
How can you tell the difference between viral, bacterial and TB meningitis from the CSF interpretation?
Bacterial: high neutrophils, high protein, low glucose
Viral: high lymphocytes, normal protein, normal glucose
TB: high lymphocytes, high protein, low glucose
113
How would you manage meningitis or meningococcal septicaemia?
Primary care - IM benzylpenicillin
| Secondary care - IV cefatriaxone (plus amoxicillin if <3 months old)
114
When would you use steroids to treat meningitis?
If there is suspected or confirmed bacterial meningitis
Give first steroid dose within 4 hours of starting antibiotics
NOTE: do not use steroids in children <3 months
115
What are the most common causes for meningitis in neonates?
Group B strep
E.Coli
Listeria Monocytogenes - this is sensitive to amoxicillin which is why you give it to children <3
116
What is the most common cause for meningitis in children aged 3 months - 5 years
Niessria meningitis
Streptococcus pneumoniae
Haemophilis influenza B
117
What is the most common causes for meningitis for children over the age of 5?
Niesseria Meningitis
| Streptococcus Pneumoniae
118
Which vaccinations are given to children to help prevent against meningitis?
Men B - protects against group B strep (given at 8, 16 weeks and 1 year)
Hib/MenC - protects against haemophilis influenza and meningitis C (given at 8, 12 and 16 weeks, and 1 year)
Prevenar 13 - protects against step pneumonia (given at 8 weeks, 16 weeks, and 1 year)
119
What should all children have after having meningitis?
Why?
An audiology assessment
This is done at 4 weeks after discharge
The risk of hearing impairment is high - bilateral hearing loss can occur in up to 4% of patients
120
What are the main gross motor developmental milestones?
```
6 months - good head control
9 months - can sit unsupported, can crawl
12 months - can stand and cruise
18 months - can walk unsupported
2 years - can jump, kick, throw
3 years - can pedal tricycle
```
121
What are the main vision and fine motor milestones?
6 weeks - can fix and follow
6 months - can transfer objects from hand to hand
1 year - effective grip
18 months - can stack 2-3 cubes
2 years - can stack 6 cubes, can draw a circle
3 years - can stack 10 cubes, can use knife and fork
122
What are the main hearing, speech and language milestones?
```
3 months - turn to sounds, coos
6 months - vocalises
9 months - babbles e.g, mama
12 months - imitates sounds (1 or 2 words)
18 months - >5 words
2 years - can say 2 word phrases
3 years - can say short sentences
```
123
What are the main social, emotional and behavioural milestones?
```
6 weeks - smiles
6 months - enjoys facial expressions
9 months - waves bye bye
12 months - plays peekaboo
18 months - can mimic actions
2 years - can use objects to play
3 years - can peer interact and role play
```
124
What is Lyme disease?
Bacterial infection cause by a tick bite - where the tick is a carrier for the bacteria borrelia burgodoferi (Not all ticks carry this bacteria)
125
What are the 3 stages of Lyme disease?
Early disease - fever, arthralgia, malaise, rash
Several weeks after - meningitis, facial palsy, arthritis
Years/months later - neuropsychiatric manifestations
126
What is the name of the rash which is commonly seen in Lyme disease?
Erythema Migrans
It is an expanding circular rash - can occur up to month after a tick bite and last for several weeks
Looks like a bulls eye
127
What is the treatment for Lyme disease?
Amoxicillin and cefuroxime
128
What are the common symptoms of UTI in children?
```
Fever
Vomiting
Irritability
Poor feeding
Failure to thrive
Loin tenderness (pyelonephritis)
```
129
What is the most common pathogen responsible for UTI in children?
E.Coli
130
If you suspect a UTI in a children under the age of 3 months, what should you do?
Urgent referral to paediatric specialist unit
Parenteral antibiotics
Urine sample sent for urgent microscopy and culture
131
How can you get a urine sample from a child?
Clean catch sample is recommended
If unobtainable can use urine collection pads
When not possible to use non invasive methods then you could use suprapubic aspiration guided by USS
132
Deepest on the microscopy results, when would you treat as a UTI and when would you not?
Both Nitrate and Leukocyte positive - UTI
Nitrate positive but leukocyte negative - UTI
Nitrate negative but leukocyte positive - only start antibiotic if clinical signs of UTI
Nitrate negative and leukocyte negative - No UTI
133
At what age would an upper UTI be more common?
In a child less than 3 months old
134
What is the antibiotic of choice for lower UTI in children?
1st line - Trimethoprim
| 2nd line - nitrofurantoin
135
What is the antibiotic of choice for upper UTI in children?
1st line - cefalexin
| 2nd line - co-amoxiclav
136
Regarding UTIs in children, when would you want to investigate further?
In children <6 months old with UTI
In children with recurrent UTIs
If any features of an atypical UTI are present
137
What defines an atypical UTI?
If the child is seriously ill
If there is poor urine flow
If there is an abdominal or bladder mass
If there is raised creatinine
In septicaemia
Failure to respond to treatment within 48 hours
Non E.Coli infection
138
What defines a recurrent UTI in children?
2 or more upper UTI
1 upper and 1 or more lower UTI
3 or more lower UTI
139
When would you want to do a renal USS in a child with a UTI?
Why?
If they are <6 months
If they have atypical UTI
To check for any hydronephrosis due to obstruction or vesicoureteric reflex
140
What is an MCUG?
| What is the use in it?
Micturating cystogram
Where you fill bladder with contrast and take x ray as child voids urine
Helps to identify any vesicoureteric reflex (VUR), OR posterior urethral valves
141
What is DMSA?
Dimercaptocuccinic acid scintigraphy
Radionucliotide scan of kidneys - isotope is given and healthy kidneys takes up isotope whereas unhealthy tissue does not
142
When would you do a DMSA and why?
If a child had recurrent UTI or atypical UTI <3 years
Carried out 4-6 months after infection to look for any renal scaring and damage which followed after infection
143
If DMSA shows scaring of kidney after infection, what are the protocols?
There is a 10% risk of developing hypertension in long term
| Child will need annual assessment of BP
144
If MCUG shows VUR on x-ray, what is the follow Up protocol in children?
Usually prophylactic antibiotic given to prevent UTI
If low grade VUR - then will resolve by age 3-4
If high grade VUR - then children are at risk of recurrent UTIs
145
What are posterior urethral valves?
Condition in boys where there is a blockage in the urethra which occurs during development
Results in one or both kidneys not forming properly
146
How may posterior urethral valves present?
May present with dribbling of urine in baby boys
May present in utero with a lack of amniotic fluid - as the majority of fluid is made up of urine
147
What is nocturnal enuresis?
What age group is it most common in?
Bedwetting
The most common type of urinary incontinence in children
5-10 year olds (tends to resolve after then)
148
How many times must a child wet their bed for a diagnosis of nocturnal enuresis to be made?
Minimum episode of once a month for at least 3 months
149
How can nocturnal enuresis be managed conservatively?
Avoid caffeine based drinks
Encourage adequate fluid intake - as fluid restriction may make it work
Treat constipation - if this is causing it
Ensure correct voiding posture
Encourage children to void every 2-3 hours during the day
150
What are the other non conservative management options for nocturnal enuresis?
Alarm training - small alarm attached to bed, if it gets wet then it sets the alarm off and wakes the child up so they can go to the toilet
Desmopressin - acts to reduce production of urine by increasing water resporption (this is a last resort)
151
Why is an early morning urine sample best for identifying kidney and bladder disease?
Urine is more concentrated in morning
| So contains higher amounts of elements and proteins
152
At what level of proteinuria would you be concerned?
>20mg/mmol - this may indicate tubular disease
>200mg/mmol - this is nephrotic ranges
ACR: >30mg/mmol
153
What is the diagnostic triad for nephrotic syndrome?
Proteinuria >35g/L in 24 hours
Hypoalbuminaemia - circulating albumin <25g/L
Odema - due to less protein in circulation so cannot keep hold of water in intracellular spaces, therefore more water leaks out into the extracellular spaces
154
What is the more common form of nephrotic syndrome in children?
Minimal change disease
155
What are the known causes of minimal change disease?
Idiopathic
NSAID use
Paraneoplastic - associated with Hodgkin's lymphoma
156
How is minimal change disease managed in children?
8 week course of oral prednisolone
157
What are the main complications of nephrotic syndrome?
Thromboembolism - due to loss of antithrombin III
Hyperlipidemia - due to loss of albumin, the liver increases more albumin and has a side effect of lipid production
Infections - due to loss of Ig from kidneys, this is more recognised in children
158
Why are children with nephrotic syndrome more at risk of sepsis?
Due to loss of antibody proteins
159
Which bacterial is most responsible for sepsis related to nephrotic syndrome in children?
Streptococci infection
160
What is the prognosis for children with nephrotic syndrome after steroid treatment?
1/3rd - no relapse
1/3rd - infrequent relapse
1/3rd - frequent relapse
161
What is renal Agenesis?
Where one kidney is either absent or underdeveloped due to hereditary renal adysplasia
162
What is renal hypoplasia?
Where one kidney is smaller than the other
163
What is a horseshoe kidney?
Where both kidneys are fused at the lower pole
164
What is the most common cause of AKI in children?
Pre renal cause
Due to intravascular fluid depletion and poor renal perfusion - where children are dehydrated or have fluid loss
165
What are the differentials for seizures in children?
```
Vasovagal syncope
Breath holding attack
Reflex asytolic syncope
Febrile convulsions
Epilepsy
Meningitis
```
166
How does vasovagal syncope present in children?
Normally occurs when a child faints in a hot and stuffy environment
Or can occur after standing too long
Think of 3 P's (Position, prodrome, provoking factors)
Clinic movements only last a few seconds
167
How would a breath holding attack present?
Precipitated by anger or crying
Toddler cannot catch their breath because of this
They may go blue, pale and stiff and limp
LOC may occur but quick recovery
168
How would reflex asytolic syncope present?
Triggered by fear, anxiety or pain (common triggers are head trauma or cold food)
Typically child becomes pale and limp, losing consciousness briefly
Followed by involuntary tonic/clinic movements
Urinary continuance may happen but NO tongue biting
Episodes last <1 minute and child makes rapid recovery
169
How do febrile convulsions present?
High temperature triggers seizure (>38) - usually following viral illness
Tonic/clonic seizure lasting <5 mins usually
170
At what age group do febrile seizures effect?
Between 6 months and 6 years of age
171
What is the difference between a simple febrile seizure and a complex febrile seizure?
Simple - <15 mins, does not reccur within 24 hours
Complex - >15 mins, recurs more than once in 24 hours, asscoatied with post neurological abnormalities
172
How would you counsel a parent whose child had a febrile seizure?
Common - effect 3% of children
Explain why it happens
30-40% may have another one - if this happens then ensure child is led down and stabilise head and wait for it to pass
If lasting >5 mins then should seek medical attention
If it recurs within 24 hours then will need hospital admission
Slight increase in risk of developing epilepsy when older
173
What is the prevalence of epilepsy in children?
0.5%
174
What are the common risk factors for developing epilepsy?
```
Birth asphyxia
Meningitis when young
Cerebral palsy
Trauma
Febrile seizures in childhood
Genetic link
```
175
What are the known triggers for epilepsy?
Watching TV
Flashing lights
Lack of sleep
176
What is status epilepticus?
Where a seizure lasts for longer than 5 minutes
177
How is status epilepticus managed?
Oxygen, check glucose
Step up after every 5 mins:
1st line - buccal midazolam or rectal diazepam
2nd line - IV lorazapam
3rd line - IV phenytoin (to be delivered by glass syringe)
178
What would indicate further investigation via CT/MRI when a child has a seizure?
Suspected raised ICP
Progressive neuroglocial deficit
In any child under the age of 2
If there is focal epilepsy
If there is associated learning difficulties
If epilepsy is resistant to full doses of 2 appropriate drugs
179
What is the inheritance pattern of the majority of inborn errors of metabolism?
Autosomal recessive
180
What is the difference between organelle disease and small molecule diseases in regards to inborn errors of metabolism?
Organelle disease - inheritance of metabolism in an organelle of the cell e.g mitochondria, lysosomes
These typically result in chronic neurodegnereation
Small molecule disease - inheritance of metabolism of a process in the cytoplasm. These are typically manageable by diet
181
What is phenylketonuria (PKU)?
Inborn error of metabolism (small molecule disease)
Screened for at newborn heel check
Lack of enzyme that converts phenylalanine to tyrosine
Leads to build up of phenylalanine (amino acid)
This can be toxic to the brain at high concentrations - can cause seizures
182
How do you manage phenylketonuria (PKU)?
Body doesn't produce this amino acid - so the only way you can get it is through diet
Therefore can manage PKU with low protein diet
To avoid being protein deficient - child can take protein supplements of all amino acids (apart from phenylalanine)
183
What is medium-chain acyl CoA dehydrogenase deficiency (MCADD)?
Lack of acyl-CoA dehydrogenase enzyme
This is responsible for breaking down fats in the body
Means that when the body is using lots of energy e.g, when they are ill, or when they are exercising, they may need this pathway
And as fats are unable to be broken down fully then it can lead to a build up of toxic metabolites
184
How is MCADD managed?
Manage catabolic episodes - ensure to take sugary drinks when ill (vomiting, diarrhoea, fever)
When vomiting replace with 10% dextrose solutions
Avoid long periods without eating during newborn period
185
How might a baby with an inborn error of metabolism present?
```
Poor feeding
Drowsiness
Sleepiness
Vomiting
Low energy
Seizures
```
186
What are the important investigations to consider in a newborn who appears drowsy after a period of good health?
```
Sugar levels
ABG
Ammonia levels
Chloride
U&Es
```
187
What are the normal ranges for ammonia in neonates and children?
Neonates <100micromols/L
| Children <50micromols/L
188
How would you work out an anion gap?
(Na+K) - (Cl+bicarbonate)
189
What is a normal anion gap?
<11
190
What are the pathological causes of hyperammonia in children?
Severe sepsis
Liver failure
Severe cardiac disease
Inborn error of metabolism
191
Where does ammonia come from?
What is is normally converted to?
It comes from amino acids as the side chain amine group
When proteins are broken down in the blood then circulating ammonia is converted into urea (less toxic form)
192
Why would you get a build up of ammonia if there was a problem with the urea cycle?
Ammonia cannot be converted to urea
193
What are the 2 inborn errors of metabolism groups which lead to hyperammonaemia?
Urea cycle disorders - ammonia cannot be converted to urea
Organic acidaemias - where there is an abnormal upstream of enzyme blockage, leading to metabolic acidosis and hyperammonemia
194
What is galactosemia?
An inborn error of metabolism that presents in the first few weeks of life
Due to lack of the enzyme galactose-1-phosphate urydial transferase (GAL-1-PUT)
Baby's cannot break down galactose
195
How is galactosemia managed?
Treat with special galactose free diet
Galactose is present in most dairy products and baby formulas
So you have to give substitutes like soy milk
196
What is the normal feeding for a newborn?
Should be feeding 150mg/kg/day
Should be feeding every 3-4 hours at least
197
How would you check with a mum if the baby is feeding okay?
Ask how long baby is feeding for - should be 5-10 mins
Does baby feel sleepy afterwards - this is normal
Ask about stool colour - stool should be passed after every feed (stool is usually dark black for first few days of life then changes to chicken korma colour)
198
What is the normal amount of weight loss for neonates?
Normal for neonates to lose up to 10% of weight in first week of life - this is because they are adjusting to breast feeding
Weight loss will stop by 3-4 days and should have regained weight by end of 2nd week
199
What are the differentials for weight loss in neonates?
```
Poor feeding e.g poor techniques
Neglect
Cystic fibrosis
Cows milk protein allergy
Congenital heart disease
Pyloric stenosis
```
200
How does cows milk protein allergy present?
```
Constipation or diarrhoea
Blood in stool
Vomiting
Weight loss
Irritability
Papular macular rash (Hives like)
Skin reactions - swelling of lips, and face around eyes
Eczema
```
201
How is cows milk protein allergy managed>
Advised to remove all cows milk from child's diet for period of time - either use special formula OR if breast feeding mum should cut out all cows milk from diet
Every 6-12 months assess to see if intolerance is gone - slowly try to introduce cows milk
Child normally grows out of it by 5 years
202
What are the common features of autism in a child?
Difficulties in communication and social interaction
Social isolation
Not knowing how to take turns
At a younger age may present with tantrums, frustration and being a difficult child
Repetitive behaviours
203
Which medical disorders are associated with autism?
Fragile X syndrome
Tuberous sclerosis
Williams syndrome
Rubella
204
How do you diagnose ADHD?
Inattention, hyperactivity and impulsivity
Must be present for at least 6 months
Must have started to show symptoms before the age of 12
Must be present in 2 or more setting (e.g, at home and at school)
205
What are the risk factors for ADHD?
```
Family history
Substance use
Psychiatric illness
Maternal smoking
Low birth weight
```
206
How is ADHD managed?
Environmental modifications is the main management
Psychoeducation - parents and child attend ADHD focus group to understand ADHD and how it presents in order to make environmental modifications
School accommodations - work to create individual education plan
(Contact SENCO - special educational needs coordinator)
Stimulant medications - e.g, Ritalin (methylphenidate) - if symptoms are still an issue after environment modifications have been made
207
What is the stepwise medical management for ADHD?
1st line - methylphenidate (Ritalin)
| 2nd line - lisdexamefetamine, dexamfetamine
208
What is cerebral palsy?
Disorder of tone, posture and movement
Cause by a non progressive brain lesion in a developing brain
This brain damage can occur before, during or soon after birth
209
When do the symptoms of cerebral palsy usually present?
In the first 2-3 years of life
210
What are the symptoms of cerebral palsy?
Delay in reaching motor milestones - e.g, not sitting by 8 months, or not walking by 18 months
Seeming too stiff or too floppy
Weak arms or legs
Fixity, jerky or clumsy movements
Walking on tip toes
Early hand preference (in first year of life)
Asymmetrical movement or posture
211
What does hemeplegic cerebral palsy mean?
Where the brain lesion only effects one hemisphere - so only the opposite side of the body is effected
This is the most common form of cerebral palsy
212
What are the causes of cerebral palsy?
Any damage to the brain before, during or after birth. These can include:
- premature birth
- infection during pregnancy
- birth hypoxia
- any pregnancy complication
- head trauma soon after birth
- meningitis in neonate
213
What is HIE?
Hypoxia ischemic encephalopathy
Where there is reduction in the supply of oxygen to the brain, due to a low blood flow to the brain. This occurs just before or after birth when an infants brain doesn't receive enough oxygen or blood
214
What is perventricular leukomalacia?
A type of brain injury that affects premature infants
Involves the death of small areas of white matter in the brain around the ventricles
Due to hypoxic/ischemic insult
Is a high risk factor for cerebral palsy
215
What are the 3 types of cerebral palsy?
Spastic (70-80%) - stiff movements, motor cortex damage
Ataxic (6%) - shaky movements, cerebellum damage
Dyskinetic (choreo-athetoid) - involuntary movements, basal ganglia damage
216
How is cerebral palsy managed?
No cure - mainly conservative management
Physiotherapy - to help with movement
Speech therapy - to help with communication and swallowing
Medications to reduce muscle tone
Surgery - to treat movement or growth problems
217
What medications are available for patients with cerebral palsy to help reduce muscle tone?
Baclofen - treats muscle spasticity
Diazepam - muscle relaxants
Botulinum toxin - muscle relaxant
218
What is muscular dystrophy?
A group of disorders where there is degeneration of the muscles leading to muscle weakness
They are a group of inherited genetic conditions
MD is a progressive condition which means it gets worse over time
219
What are dystrophinopathies?
They are the most common type muscular dystrophy
Due to a mutation in the dystrophin gene - this is an important protein link in muscles (in the absence of the gene muscle cells destabilise and die)
220
What are the 2 dystrophinopathies?
What is there genetic inheritance?
Duchenne muscular dystrophy (DMD)
Becker muscular dystrophy (BMD)
X-linked recessive - so most commonly present in males
221
What is the difference between DMD and BMD?
DMD - mutation in dystrophin so there is no protein production. This is much more severe and symptoms occur by the age of 5
BMD - mutation in dystrophin so there is protein production but it is misformed. This is a milder form of DMD. Symptoms present later between the age of 10-20
222
What are the signs and symptoms of muscular dystrophy?
Late motor development
Tie toeing gait
Calf pseudohypertrophy - large calves (due to fat and firboids)
Gowers sign
223
What is gowers sign?
When a child has to use there hands to help them up from a squatting to standing position due to lack of thigh muscle strength
224
How is muscular dystrophy diagnosed?
Genetic testing - look for mutations in dystrophin
Creatine kinase (CPK) - this may be raised due to muscle breakdown
Muscle biopsy - to stain for dystrophin protein
225
How is muscular dystrophy managed?
No cure
Steroids - can sometimes slow degeneration (but be aware of side effects of long term steroids)
Physiotherapy - to help with symptom management
226
What is global development delay?
When a child has significant delay in the milestones of two or more core areas?
227
Which disease are associated with global developmental delay?
Down's syndrome
| Tuberous sclerosis
228
What is tuberous sclerosis?
Rare genetic conditions
Causes benign tumours to develop in different parts the body
Presents with developmental delays, seizures, disability
Can also present with non cancerous growth on brain, skin, kidneys, heart, eyes and lungs
229
What is the glucose sugar levels needed to diagnose type 1 diabetes?
fasting glucose >7
| 2 hour plasma glucose >11
230
When would you consider type II diabetes in children?
If they have strong family history
In obese children
Black or asian descent
Acanthosis nigricans
231
What are acanthosis nigricans?
What are the causes?
Black hyperpigmentation found in the skin folds
Type II diabetes
Cushings, PCOS, hypothyroidism
Steroid treatment
Stomach cancer
232
What is C-Peptide?
Involved in the production of insulin
When pancreas cells make insulin they release C-peptide as a by product
Therefore it will only be present in type II diabetes as in type I diabetes no insulin is produced
233
What are GAD antibodies?
Antibodies present with instruct the immune system to destroy pancreatic insulin producing cells
They are only present in type I diabetes
234
How is type I diabetes managed?
Insulin therapy
Can either have multiple injections - one daily long acting insulin, as well as short acting insulin before meals
Or can have insulin pump therapy - continuous device that infuses insulin
235
What are the blood glucose targets for type I diabetics?
On waking - 4-7
Before meals - 4-7
After meals 5-9
While driving >5
236
When should type I diabetics monitor their blood glucose levels?
Should perform at least 5 capillary blood tests per day
| Should monitor more frequently during intercurrent illness
237
What are the diabetic sick day rules?
Keep taking insulin even if you are not eating
Check blood sugar every 4 hours - even during the night time
Stay hydrated and drink plenty of unsweetened drinks
238
How many times should HbA1c be measured in type I diabetes?
4 times a year
239
How is mild hypoglycaemia managed?
Encourage fast acting glucose solution (liquid carbohydrate form)
Recheck BMs in 15 mins
Repeat fast acting glucose if it persists
As symptoms improve, give oral complex long acting carbohydrate to maintain glucose levels
240
How is severe hypoglycaemia managed?
IV 10% glucose - if IV available
IM glucagon - if IV not available
Oral glucose solution (glucogel) - if they are fairly conscious
241
What are the different IM glucagon doses for the different ages and weights?
> 8 years (or >25kg) - 1mg glucagon
| < 8 years (or <25kg) - 500micrograms glucagon
242
Why do type 1 diabetes have to be careful when drinking alcohol?
Can cause hypoglycaemia
Diabetics must eat carbohydrate rich foods before and after drinking alcohol
Diabetics must monitor blood glucose regularly when drinking alcohol
243
How does hypoglycaemia caused by alcohol need to be managed and why?
IV glucose
As IM glucagon may be ineffective in alcohol induced hypoglycaemia
244
What are the long term complications of type 1 diabetes and how are these monitored?
Thyroid disease - check for at diagnosis and annually
Diabetic retinopathy - check annually from 12 years with eye screen
Kidney disease - check ACR annually from 12 years
Hypertension - check annually from 12 years
245
What is diabetic ketoacidosis (DKA)?
When there is a severe lack of insulin so the body cannot use intracellular glucose
Therefore it uses fats as an alternative energy source - the breakdown product of fats is ketones
Ketones are poisonous chemicals which when built up can cause acidosis in the body
246
When is a DKA most likely to occur in children?
At diagnosis - many type 1 diabetes present with this
When child is ill - as they have poor controlled insulin levels
During growth spurt
If they stop taking insulin for some reason
In patients with insulin pumps if the cannula dislodges
247
How does a DKA present?
```
Nausea or vomiting
Abdominal pain
Hyperventilation - respiratory compensation for acidosis
Dehydration
Reduced consciousness level
```
248
What is kussmaul breathing?
Deep and laboured breathing associated with severe metabolic acidosis
The body is trying to compensate for the acidosis by blowing off CO2
It is often associated with DKA and kidney failure
249
Is hyperglycaemia always present in DKA?
NO
You should always suspect DKA in a symptomatic patient even if their BMs are not elevated
250
How would you investigate someone with suspected DKA?
Capillary blood glucose - >11 then this is suggestive
Capillary blood ketones (beta-hydroxybutyrate) - if >3 then this is suggestive
ABG - to check for metabolic acidosis
U&ES - to check for sodium, potassium, urea and creatine plasma bicarbonate
251
When would you diagnose someone with DKA?
If any of the following are present:
Ph <7.3
Bicarbonate <18
Beta-hydroxybutyrate (ketones) >3
252
At what ph level would you diagnose severe DKA?
Ph <7.1
253
What is the immediate management for a person with suspected DKA?
Transfer to a recognised paediatric high dependancy unit
Insulin
Fluids
254
Why would you not give an intravenous fluid bolus to a child with a DKA?
As faster rehydration of associated with an increased risk of cerebral oedema
Therefore you must aim to replace the fluid deficit evenly over the first 48 hours
255
How do you decide whether to give subcutaneous insulin and oral fluids as opposed to IV insulin and IV fluids?
If the child is alert, and not vomiting or dehydrated - can go for subcutaneous insulin and oral fluids
If the child has decreased consciousness, is vomiting and is clinically dehydrated - then you give IV insulin and IV fluids
256
What IV fluids do you give to someone with DKA?
0.9% Sodium chloride with 40mmol potassium chloride (WITHOUT glucose) - use until plasma glucose is below 14
Once glucose falls below 14 - then you can change to 0.9% sodium chloride with 5% glucose and 40mmol potassium chloride
257
How much insulin would you give IV to someone with DKA?
When would you start insulin infusion
IV infusion of 0.05-0.1 units/kg/hour
Start insulin within 1-2 hours after beginning IV fluids
258
What must you remember when switching IV insulin to subcutaneous or pump insulin?
To start subcutaneous insulin at least 30 mins before stopping IV insulin
To restart insulin pump at least 60 mins before stopping IV insulin
259
If someone with DKA presents with reduced consciousness, what would you consider?
Nasogastric tube - to reduce risk of aspiration due to vomiting
Intubation - if they cannot protect airway
Inotropes (e.g, adrenaline) - if they are in hypertensive shock
260
What are the symptoms of cerebral oedema?
```
Headache
Agitation
Irritability
Unexpected fall in HR
Increased BP
Falls in respiration
```
261
What is the management of cerebral odema?
Mannitol - 20% 0.5-1g/kg over 10-15 minutes
262
What are the risks of insulin in DKA management?
Can cause hypokalaemia
If potassium is <3 then think about temporarily stopping insulin infusion
263
Why is important to measure U&Es during DKA management?
To measure potassium levels - as insulin can cause hypokalaemia
264
How would you counsel a parent on the MMR vaccine?
Given at 12 months and 3-4 years
Protects against Measles, mumps and rubella viruses
These can have serious complications such as meningitis, encephalitis
Vaccine contains weakened versions of the viruses that trigger immune system to produce antibodies
Link to autism - one study in 1998 - has been discredited - and there is no known link
Side effects - developing mild form of measles or mumps (lasting a few days maximum) (not infective)
In rare cases may develop rash - see GP if this occurs
You cannot infect someone else with MMR vaccine
265
What is testicular torsion?
Twisting of the spermatic cord within the scrotum
This cord contains vessels which supply blood to the scrotum
Twisting leads to reduced blood flow and can cause sudden pain and swelling
266
What are the symptoms of testicular torsion?
```
Sudden sever pain in scrotum
Swelling of scrotum
Abdominal pain
Nausea and vomiting
Frequent urination
Fever
```
267
At what age is testicular torsion most common?
Between the ages of 12 and 18
268
How is testicular torsion managed?
Emergency surgery - to save testicle
269
What is henoch-schonlein purpura (HSP)
Vasculitis commonly occurring in children
Causes a rash on lower limbs
Not usually serious
But sometimes can lead to kidney problems
270
What are the symptoms of henoch-schonlein purpura (HSP)?
```
Purpuric rash on legs or bottom
Abdominal pain
Diffuse joint pain - in knees or ankles
Scrotal pain - may occur in 13% of boys
Haematuria
```
271
What are the risk factors for HSP?
Male sex
Aged 3-15 years
Previous URTI
272
How is HSP managed?
Most cases of HSP resolve spontaneously within 4 weeks so management is purely symptomatic:
Analgesia - for abdominal pain
Oral prednisolone - for scrotal involvement or severe oedema or abdominal pain
IV corticosteroids - if pain is accompanied by nausea and vomiting
273
If a child with HSP is clinically stable, how would you advise the parents to manage at home?
Pain relief for symptoms
| Educate on urine dip - contact if there is +++ protein or blood
274
What investigations should you do in a child with HSP and why?
U&Es
ACR
As HSP can cause renal damage
275
When should children with HSP be followed up until?
Followed up for 6 months with urinalysis and BP monitoring
276
If a child with HSP had abnormal urinalysis then what would you want to do?
PCR - check creatinine to assess renal function
277
If a child with HSP had persistent proteinuria or renal insufficiency what would you do?
Referral to nephrologist
278
What is immune thrombocytopenic purpura?
Disorder of low platelets
Where the blood doesn't clot due to low platelets
The immune system produces antibodies against platelets
279
When does ITP occur in children?
Usually occurs acutely following an illness
280
What are the symptoms of ITP?
Bruising easily
Petechiae rash
Spontaneous bleeding - nosebleeds, bleeding from gums
Blood in urine or stool
281
How is ITP managed?
In children it usually self resolves in 6 months
However if medication is required you can give:
Prednisolone - which helps to increase platelet count by decreasing the activity of the immune system
Intravenous immunoglobulin - given when you need to increase platelet count quickly e.g, in Cristal bleeding
Rituximab - antibody therapy that targets the immune cells responsible for producing the proteins that attack platelets
282
What should you suspect in anaemic children?
Nutrition deficiencies - this is common in children
283
At what age does sickle cell disease present and why?
After 6 months
This is because there is a loss of fetal haemoglobin and production of sickle cell haemoglobin
284
What is the pathophysiology in sickle cell disease
Genetic disease
RBCs become sickle shaped so are more likely to get stuck in small blood vessels
This can cause pain
Also increases risk of infection - as cells are unable to travel in blood
285
What is the biggest risk to children with sickle cell anaemia?
How is this managed?
Risk of infection
Vaccinations
Antibiotic prophylaxis - in children under 5 years
286
How is sickle cell disease managed long term?
```
Pain management (Acute and chronic)
Prompt treatment of investinos
Management of acute chest syndrome
Prevention of stroke
Prevention of chronic organ damage (kidney, renal, pulmonary)
```
287
Can sickle cell disease be cured?
Yes
By bone marrow transplantation
288
What are some treatments used in sickle cell disease?
Hydroxycarbamide - to increase the amount of fetal haemoglobin
Blood transfusion - in order to keep HbS below 30%
289
What bacteria are those with sickle cell disease most at risk of getting?
Pneumococcal bacteria
290
What is the most common type of childhood cancer?
Acute lymphoblastic leukaemia (ALL)
291
How does ALL present
```
Bone/joint pain
Loss of appetite
Bruising, nosebleeds, bleeding gums, petechiae
Lymphadenopathy
Recurrent infections
Anaemia
```
292
What is Ewigs Sarcoma?
Rare childhood cancer what develops in the bone
Occurs mainly in teenage years and is more prevelent in boys
293
What is neuroblastoma?
At what age does it present?
Cancer of the neural crest cells
Cancer most commonly occurs in the adrenal glands
Generally presents in early childhood before the age of 5
294
At what age does retinoblastoma usually present?
In children under the age of 5
295
What are the symptoms of retinoblastoma?
White pupil - white colour behind pupil that does not reflect light (known as white reflex)
Crossed eyes - squint or abnormal eye movement
Red eye - due to painful irritation that may persist if tumour is large
296
What is wilms tumour?
What age does it commonly occur?
Cancer of the kidney in children
Occurs between the age of 3-4 years
297
What are the causes for neonatal jaundice?
Premature birth - as babies cannot process bilirubin
Significant bruising during birth - breakdown of RBCs
Breast feeding - breast fed babies higher risk of jaundice
Haemolysis - due to internal bleeding or infection
Liver malfunction
Enzyme deficiency
298
What is the risk of having a high bilirubin in a baby?
Encephalopathy
As high levels of bilirubin are toxic to the brain
Kericternus can occur if bilirubin encephalopathy causes percent damage to the brain
299
How would you counsel a parent whose child has been born with Down's syndrome?
Explain - extra chromosome because of change in the sperm or the egg before birth. This happens by chance
Risk - increasing age
Prognosis - level of learning disability, everyone is different. People from downs are not ill. Life expectancy 50 years
Risk of heart defects - these can be fixed at birth
Long term health problems risk - hypothyroidism, celiac disease, problems with vision or hearing, sleep apnea
More information: Down's syndrome association
