Paeds Flashcards

1
Q

what are 3 characteristic signs of pneumonia on examination?

A

bronchial breath sounds
focal coarse crackles
dullness to percussion

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2
Q

what is the most common cause of pneumonia in children?

A

streptococcus pneumonia

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3
Q

what is the most common viral cause of pneumonia?

A

respiratory syncytial virus (RSV)

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4
Q

what is the typical x-ray finding in s. aureus pneumonia?

A

pneumatocoeles (round air filled cavities) and multilobe consolidation

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5
Q

what are 2 bacteria that are more likely to cause pneumonia in pre/un-vacinated children?

A

GBS
Haemophilus influenza

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6
Q

which bacterial pneumonia can cause extrapulmonary manifestations and what are they?

A

Mycoplasma pneumonia
Can cause erythema multiforme

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7
Q

what is the management of pneumonia?

A

1st - amoxicillin

+/- macrolide (erythro, clarithro, azithro) for atypicals or allergy

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8
Q

what is the name of the condition where people cannot convert IgM to IgG so cannot form long term immunity?

A

immunoglobulin class-switch recombination deficiency

VACCINES DONT WORK!

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9
Q

what age group is typically affected by croup?

A

6 months - 2years

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10
Q

what is the pathophysiology of croup?

A

upper resp tract Ix causing oedema of the larynx

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11
Q

what is the most common pathogenic cause of croup?

A

parainfluenza virus

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12
Q

what are 2 key features of croup caused by parainfluenza virus?

A

improves in <48 hours
responds well to dexamethasone

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13
Q

what are 3 other common pathogenic causes of croup

A

influenza
adenovirus
RSV

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14
Q

what is a possible cause of croup especially in pre/unvaxinated children?

A

diphtheria - leads to epiglottitis!!! high mortality!

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15
Q

what are 5 symptoms of croup?

A

increased work of breathing
barking cough in clusters
hoarse voice
stridor
low grade fever

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16
Q

what is the treatment for croup?

A

supportive

oral dexamethasone - single dose 150 mcg/Kg (can repeat at 12 hours)

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17
Q

what is the stepwise management of severe croup?

A

oral dexamethasone
O2
Neb budesonide
Neb adrenaline
Intubation and ventilation

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18
Q

what can be seen on x-ray in croup?

A

Steeple sign - tracheal narrowing

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19
Q

what age is virally induced wheeze typical in?

A

<3 years

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20
Q

what is the pathophysiology of viral induced wheeze?

A

viral infection causes inflammation and oedema which reduces space for air flow in a greater proportion due to small size of child’s airways- Poiseuilles law

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21
Q

what is the management of viral induced wheeze?

A

same as acute asthma

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22
Q

what kind of wheeze is heard in asthma and viral wheeze?

A

polyphonic expiratory wheeze throughout

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23
Q

what is moderate asthma?

A

peak flow >50% predicted
normal speech
otherwise well

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24
Q

what is severe asthma? (6)

A

peak flow 33-50% predicted
sats <92% (different to adults)
unable to complete sentences/feed
Use of accessory muscles

RR >40 1-5years, >30 5+ years
HR >140 1-5 years, >125 5+ years

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25
what is life threatening asthma?
peak flow <33% predicted sats <92% exhaustion and poor resp effort hypotension silent chest cyanosis altered consciousness/confusion
26
what is a physical finding on the chest wall in severe chronic asthma?
harrison sulus - indentation in chest wall along 6th rib can be bilateral or unilateral
27
what is the acute management of asthma/viral wheeze?
Escalating Bronchodilators - Salbutamol, ipratropium bromide, magnesium sulphate, aminophylline PLUS - Steroids - prednisone (oral) or Hydrocortisone IV ABx - if suspected
28
what is the stepwise progression of bronchodilators in acute asthma?
IHR/Neb salbutamol (10 puffs every 2 hours or repeat nebs every 20-30 mins) IHR/Neb ipratropium bromide IV magnesium sulphate IV aminophylline Back to back nebs = 3 salbutamol then 1 ipratropium
29
what on spirometry suggests an obstructive picture?
reversibility FEV1 <80%, FEV1:FVC <0.7
30
How is asthma investigated in children 5-12 years?
1 - FeNO >35 ppb 2 - bronchodilator reversibility >12% pre-dilation reading or >0% of predicted 3 - PEF 20% variability in reading over 2 weeks 4 - skin prink testing, measing IgE
31
what is the long term management of asthma age 5-12?
1 - SABA + ICS BD 3 - SABA + ICS + LTRA (leukotriene receptor antagonist - montelukast) 4 - SABA + ICS + LABA (-LTRA) 5 - SABA + MART (low dose ICS) 6 - SABA + moderate dose ICS MART 7 - SABA + high dose ICE OR Additional drug OR Refer
32
what is the stepwise management of children <5 which chronic asthma?
1 - SABA + 8-12 week trial low dose ICS BD 3 - SABA + low dose ICS + LTRA 4 - Stop LTRA and refer
33
what is considered paediatric high dose ICS?
>400 micrograms budesonide or equivalent
34
what is considered paediatric low dose ICS?
<200 micrograms of budesonide or equivalent
35
which LABA can be used as a short acting agonist also?
folmeterol
36
can ICS cause restricted growth?
can reduced adult hight with long term use by up to 1cm . dose dependent effect
37
what is the most common cause of bronchiolitis?
Respiratory syncytial virus (RSV)
38
in what age group does bronchiolitis occur?
<1 year, most common <6 months can be up to 2 years
39
what are 5 risk factors for bronchiolitis?
Underlying respiratory disease congenital heart defects seasonal immunodeficiency prematurity
40
what are 8 typical signs of bronchiolitis?
coryzal symptoms - URTI signs of resp distress dyspnoea tachypnoea poor feeding mild fever apnoea wheeze and crackles
41
what are 8 signs of respiratory distress?
raised RR use of accessory muscles intercostal and subcostal recession nasal flaring head bobbing tracheal tugging cyanosis abnormal airway noise
42
what is the typical course of RSV bronchiolitis?
coryzal symptoms chest symptoms 1-2 days later worst day 3-4 symptoms usually last 7-10 days
43
what are reasons for admission in bronchiolitis?
<3 months, pre-existing conditions <50-70% of normal milk intake clinical dehydration RR >70 O2 <92% OA resp distress apnoea parent not able to manage/access medical help at home
44
what is the management of bronchiolitis?
ensure adequate intake - NG, IVs saline nasal drops, nasal suctioning O2 ventilation support
45
2 signs of poor ventilation of a cap gas?
rising pCO2 falling pH
46
what can be given as prevention of RSV infection?
Palivizumab monthly injection as prevention of bronchiolitis caused by RSV given to high risk babies. Provides passive protection
47
what are 4 indications for RSV prophylaxis in babies?
Chronic lung disease - requiring O2 post 28 days Respiratory disease Congenital heart disease Severe combined immunodeficiency syndrome
48
what age does laryngomalacia occur at?
infants peaking at 6 months - inspiratory stridor exacerbated by lying, feeding, upset => no coinciding resp distress problem resolves as larynx matures - around 1.5 years
49
what infection typically causes epiglottitis?
Haemophilus influenzae B
50
what are 8 symptoms of epiglottitis?
sore throat/pain swallowing stridor drooling tripod position high fever muffled voice scared and quiet septic and unwell
51
what investigation can be done in suspected epiglottitis?
lateral xray of neck - shows thumb sign or thumbprint sign excludes foreign body aspiration
52
what is the management of epiglottitis?
alert senior paediatrician and anaesthetist nebulised adrenaline - may be used prior to intubation to reduce laryngeal oedema ABx - cefriaxone steroids - dexamethasone
53
what is a complication of epiglottitis?
epiglottic abscess
54
what is chronic lung disease of prematurity (bronchopulmonary dysplasia)?
typically <28 weeks gestation suffer from resp distress syndrome and require O2 or intubation and ventilation at birth. Diagnosed on CXR when infant required O2 >36 weeks gestational age
55
what are 5 features of chronic lung disease of prematurity?
low O2 sats increased work of breathing poor feeding and weight gain crackles and wheeze increased susceptibility to infection
56
what can reduce risk of chronic lung disease of prematurity?
corticosteroids (betamethasone) in premature labour mothers <36 weeks CPAP rather than intubation Caffeine to stimulate resp effort avoid over-oxygenating
57
what is the most common pathogen of otitis media?
strep pneumoniae
58
what is the typical presentation of otitis media?
ear pain reduced hearing symptoms of URTI
59
what is the first line Abx for otitis media?
1 - amoxicillin 5 days erythro, clarithro
60
what is glue ear?
otitis media with effusions - middle ear become full of fluid due to a blockage in the eustacion tube causing loss of hearing
61
what can be seen on otoscopy in glue ear?
dull tympanic membrane with air bubbles or a visible fluid level can look normal
62
what is the natural course of glue ear?
usually resolves within 3 months without treatment
63
how long does it usually take grommets to fall out?
1 year
64
what are 3 common congenital causes of deafness?
maternal rubella or CMV genetic deafness associated syndromes - downs
65
what are 2 common perinatal causes of deafness?
prematurity hypoxia during or after birth
66
what are 4 common post natal causes of deafness?
jaundice meningitis and encephalitis otitis media or glue ear chemo
67
what is the range of normal hearing on an audiogram?
all readings between 0-20 dB
68
what are 3 symptoms additional to tonsillitis symptoms that can indicate quinsy?
trismus - unable to open mouth change in voice (hot potato voice) swelling and erythema surrounding tonsils
69
what is the most common pathogen in quinsy?
strep pyogenes (A) and Haemophilus influenzae
70
what is the management of quinsy?
drainage and Abx (co-amox) ?dex
71
what is preorbital cellulitis?
infection of upper and lower eyelid which causes red how skin swelling around eyelid and eye - distinguish from orbital cellulitis with CT scan usually no pain or reduction in eye movements, no change in vision or abnormal pupillary response
72
what is the management of preorbital cellulitis?
systemic Abx
73
what is squint also known as?
strabismus - misalignment of the eyes causing double vision
74
what are 5 causes of squint?
idiopathic hydrocephalus cerebral palsy space occupying lesion trauma
75
what are 2 tests for squint?
cover test herschberg's test
76
what is the management of squint?
occlusive patch or atropine drops in good eye
77
what are the 3 foetal circulation shunts?
ductus venosus foramen ovalae ductus arteriosus
78
what is the ductus venosus?
shunt connecting umbilical vein to inferior vena cava allowing foetal blood to bypass liver
79
what is the foramen ovale?
shunt connecting R to L atrium which allows blood to bypass R ventricle and pulmonary circulation
80
what is the ductus arteriosus?
shunt connecting pulmonary artery and aorta which allows blood to bypass the pulmonary circulation
81
are atrial septal defects cyanotic?
no - unless R sided pressure becomes so great due to pulmonary hypertension the shunt reverses - EISENMENGER SYNDROME
82
what is eisenmenger syndrome?
when a shunt reverses due to pulmonary HTN causing R sided pressure > L sided pressure leading to pulmonary circulation bypass and cyanosis. develops after 1-2 years with large shunts and in adulthood with small
83
what are 4 complications of atrial septal defects?
stroke - with DVT - clot bypasses lungs AF or atrial flutter Pulmonary hypertension and r sided HF Eismenger syndrome
84
what are 3 types of atrial septal defect?
Ostium secondum - septum secondum fails to fully close patent foramen ovale ostium primum - septum primum fails to close - tends to causes Av valve defects also
85
what is the murmur in ASD?
mid systolic crescendo-decrescendo murmur loudest at upper left sternal border with a fixed split second heart sound
86
what are 5 typical ASD symptoms?
SOB difficulty feeding poor eight gain LRTIs complications - heart failure, stroke
87
what is the management of ASD?
Referral to paediatric cardio Watchful waiting if small Transvenous catheter closure or open heart surgery
88
what can be seen on examination in eisenmengers syndrome?
cyanosis clubbing dyspnoea plethoric complection (due to polycythaemia)
89
what is the management of eisenmengers syndrome?
Heart lung transplant Management of pulmonary HTN, polycythaemia and thrombosis
90
what 2 genetic conditions are associated with VSDs?
Downs Turners
91
what 3 defects can cause eisenmenger's syndrome?
ASD VSD patent duct arteriosus
92
what is the murmur is VSD?
pan-systolic more prominent at left lower sternal border in 3/4 intercostal space. May be systolic thrill
93
what are the 3 causes of pan-systolic murmur?
VSD mitral regurg tricuspid regurg
94
what is the management for VSD?
Watch and wait if small Surgical repair - transvenous catheter closure or open heart surgery
95
what are 4 complications of VSD?
Eisenmenger syndrome Heart failure endocarditis pulmonary hypertension
96
how long does it usually take for the ductus arteriosus to close?
1-3 days to stop functioning, 2-3 weeks to fully close
97
what kind of shunt is patent duct arteriosus?
left to right - from aorta to pulmonary vessels
98
what murmur is heard in patent duct arteriosus?
continuous crescendo -decrescendo machinery murmur
99
what causes an ejection systolic murmur? (3)
aortic stenosis pulmonary stenosis hypertrophic obstructive cardiomyopathy
100
what 2 conditions that cause cyanotic heart disease?
transposition of the great arteries Tetralogy of fallot Also Eisenmengers transformation
101
what 4 conditions are coarctation of the aorta associated with?
turners syndrome bicuspid aortic valve berry aneurysm neurofibromatosis
102
what is the presentation of coarctation of the aorta?
weak femoral pulses - upper limb BP higher than lower limb - radiofemoral delay Systolic murmur below left clavicle, may radiate to scapula tachypnoea poor feeding grey, floppy baby
103
what is the management of coarctation of the aorta?
surgery - bypass graft, baloon angioplasty, stenting in high risk - prostaglandin E to keep ductus arteriosus open till surgery
104
what cardiac condition causes cyanosis at birth?
transposition of the great arteries
105
what is transposition of the great arteries?
the attachments of the aorta and pumonary trunk are swapped so R ventricle pumps blood into aorta and L ventricle into pulmonary artery
106
what is the management of transposition of the great arteries?
prostaglandins E1 - maintain ductus arteriosus Balloon septostomy - insert balloon catheter into foramen ovale to create largeASD open heart surgery - arterial switch
107
what are the 4 pathologies in tetralogy of fallot?
Pulmonary valve stenosis Right ventricular hypertrophy Overriding Aorta VSD PROVe
108
what kind of shunt is there in tetralogy of fallot?
left to right - cyanotic
109
what are 4 risk factors for tetralogy of fallot?
rubella infection increased age of mother alcohol in pregnancy diabetic mother
110
what investigations can be used for septal defects?
echo + doppler flow studies
111
what can be seen on chest x-ray in tetralogy of fallot?
boot shaped heart due to right ventricular hypertrophy
112
what are 6 manifestations of tetralogy of fallot?
cyanosis clubbing poor feeding poor weight gain ejection systolic murmur loudest in pulmonary area tet spells
113
what are tet spells?
seen in tetralogy of fallot where shunt becomes temporarily worse causing cyanotic episode - usually during waking, exertion, crying
114
what is the management of tet spells?
squatting or pulling knees to chest - increases systemic vascular resistance O2 beta blockers - relax R ventricle IV fluids morphine - decrease resp drive sodium bicarb - buffer metabolic acidosis phenylephrine infusion - increase systemic vascular resistance
115
what is the management of tetralogy of fallot?
Prostaglandin E1 - maintain ductus arteriosus Beta blockers - may be used for tet spells total surgical repair at 3-6 months
116
what is ebstein's anomaly?
R tricuspid valve lower than normal causing R ventricle to be smaller causing poor flow to pulmonary vessels - usually presents after closing of ductus arteriosus
117
what 4 conditions are associated with pulmonary valve stenosis?
tetralogy of fallot william syndrome noonan syndrome and turners syndrome congenital rubella syndrome
118
what is rheumatic fever?
autoimmune condition triggered by antibodies to group A beta-haemolytic strep - typically strep pyogenes causing tonsilitis. The process usually occurs 2-4 weeks after initial infection
119
what is the presentation of rheumatic fever? (7)
fever joint pain - migratory of large joints erythema marginatum rash shortness of breath chorea firm painless nodules carditis - tachy/brady, murmurs, pericardial rub, heart failure
120
what are 3 investigations for rheumatic fever?
throat swab and culture Anti streptococcal antibodies (ASO) titres ECHO, ECG, CXR
121
what criteria is used to diagnose rheumatic fever?
jones criteria
122
what is the jones criteria?
for diagnosing rheumatic fever TWO of JONES Joint arthritis Organ inflammation (carditis) Nodules Erythema marginatum rash Sydenham chorea OR One JONES and TWO FEAR Fever ECG changes (prolonged PR) Arthralgia Raised inflammatory markers
123
what is the management of rheumatic fever?
Abx - Benzathine benzylpenicilin or phenoxymethylpenicillin NSAIDs or salicates (joint pain) Aspirin and steroids - carditis Heartfailure - Diuretics +/- ACEi Chorea - carbamazepine
124
what are 3 complications of rheumatic fever?
recurrence valvular heart disease - mitral stenosis chronic heart failure
125
what are 2 risk factors for GORD in children?
prem delivery neurological disorders
126
what are 6 signs of problematic reflux in babies?
chronic cough hoarse cry distress after feeding reluctance to feed pneumonia poor weight gain
127
what advice can be given for GORD in babies?
small frequent meals burping regularly not overfeeding keep baby upright after feeding
128
what kind of vomiting is present in GORD?
effortless mainly after feeding
128
What volume should formula fed infants be consuming per day?
150 ml/Kg per day
129
what 3 treatments can be given in babies with GORD?
1 - breast - technique, positioning, attachment assessment 2 - breast - gaviscon (alginate) trial for 2 weeks at a time 1 - bottle - assess volume of feeds, trial smaller more frequent 2 - bottle - thickened formula 3 - bottle - Gaviscon (alginate) 3/4 - breast/bottle - PPI or H2 antagonist 4 week trial
130
what is sandifer's syndrome?
brief episodes of abnormal movements associated with GORD leading to torticollis and dystonia
131
what is a key feature of pyloric stenosis?
projectile vomiting
132
when does pyloric stenosis present?
first few weeks (4-6 weeks) of life baby failing to thrive with projectile vomiting 30 mins after feeding
133
what 3 things can be found on examination of a baby with pyloric stenosis?
observation of stomach peristalsis firm round 'olive like' mass in upper abdomen hypochloric (low Cl-), hypokalaemia metabolic alkalosis on blood gas
134
how is pyloric stenosis dianosed?
Abdo USS
135
what is the management of pyloric stenosis?
laparoscopic pyloromyotomy - Ramstedt's operation
136
what are 2 common viral causes of gastroenteritits?
rotavirus norovirus
137
which e.coli produces shiga toxin?
E.coli 0157
138
what does shiga toxin cause?
abdo cramps, bloody diarrhoea, vomiting - can lead to haemolytic uraemic syndrome
139
what is the most common cause of bacterial gastroenteritis?
campylobacter jejuni
140
where does campylobacter come from?
raw/improperly cooked poultry untreated water unpasteurised milk
141
what is the treatment of campylobacter?
azithromycin or ciprofloxacin
142
which bacteria causes gastroenteritis after food has not been refrigerated quickly (rice)?
bacillus cereus - diarrhoea resolving in 24 hours
143
what is the treatment of Giardiasis?
metronidazole
144
what are 4 possible complications of gastroenteritis?
lactose intolerance IBS reactive arthritis GBS
145
what is toddlers diarrhoea?
chronic watery diarrhoea in well <5 year olds which can often be helped by changes in diet
146
what are the 4fs of toddler's diets?
fat - shouldnt have low fat diet Fluid - not sugary drinks, not too much water Fruit + fruit juice - gut cannot absorb fructose or sorbitol easily Fibre - 12-18g per day
147
what is persistent diarrhoea?
>2 weeks
148
what is chronic diarrhoea?
>4 weeks
149
what is encopresis?
foecal incontinence
150
at what age does faecal incontinence become pathological?
4 years
151
what are 6 causes of faecal incontinence?
spina bifida hirschprungs disease cerebral palsy overflow incontinence - stress, abuse Constipation with overflow Learning disability
152
what are 5 lifestyle factors that may contribute to constipation in children?
habitual not opening bowels low fibre diet poor fluid intake sedentary lifestyle psychosocial problems
153
what are 8 secondary causes of constipation in children?
hirschprungs disease CF Hypothyroid Spinal cord lesions sexual abuse Intestinal obstruction anal stenosis cow milk intolerance
154
what are 8 red flag symptoms of constipation in children?
Failure to pass meconium neurological signs or symptoms Vomiting Ribbon stool Abnormal anus Abnormal lower back/buttocks - spina bifida Failure to thrive Acute sever abdo pain/bleeding
155
what is the first line laxative in children?
1 - Macrogol (movicol) 1-11 months - 0.5 sachets 1-5 years - 1 sachet 6-11 years - 2 sachets MAX 4 sachets 2 - add stimulant laxitive
156
what is meckel's divertculum?
malformation of distal ileum present in 2% of population that can bleed, become inflamed, rupture or cause volvutus, intussusception or obstruction
157
what is the presentation of Meckel's diverticulum?
abdo pain mimicking appendicitis Rectal bleeding - most common cause of massive GI bleed in 1-2 year olds Intestinal obstruction - volvulus and intussusception
158
what investigations can be used for meckel's diverticulum?
Meckels scan - technetium-99m pertechnetate Mesenteric arteriography
159
what is the management of symptomatic Meckel's diverticulum?
surgical resection or diverticulectomy
160
what are the 5 key features of Crohn's? (mneumonic)
NESTS No blood or mucus (less than UC) Entire GI tract Skip lesions Terminal ileum most affected + Trans mural Smoking risk factor weight loss, strictures, fistulae
161
what are the 7 key features of UC? (mneumonic)
CLOSEUP Continuous inflammation Limited to colon and rectum Only superficial mucosa Smoking protective Excrete blood and mucus Use aminosalicylates Primary sclerosing cholangitis
162
what is one stool test that can be used for IBD but only in adults?
faecal calprotectin Aged 16-40 years
163
what is the most common congenital abnormality of the small bowel?
meckel's diverticulum
164
when do people usually present with meckel's diverticulum?
<2 years
165
what is faltering growth?
fall in weight across: 1+ centiles if birth weight below 9th 2+ centiles if birth weight between 9th-91st 3+ centiles if birth weight 91+ centiles
166
what are 5 causes of failure to thrive?
inadequate nutritional intake difficulty feeding malabsorption increased energy requirements inability to process nutrition
167
what are 5 causes of inadequate nutritional intake in children failing to thrive?
maternal malabsorption if breastfeeding IDA family or parental problems neglect availability of food
168
what are 4 causes of difficulty feeding?
poor suck (cerebral palsy) cleft lip/palate genetic conditions w/ abnormal face structure pyloric stenosis
169
what are 5 causes of malabsorption in children failing to thrive?
CF coeliac disease cows milk intolerance chronic diarrhoea IBD
170
what are 4 causes of increased energy requirements in children failing to thrive?
Hyperthroidism chronic disease - CF, CHD malignancy chronic infections - HIV, immunodeficiency
171
what are 2 causes of inability to process nutrients in children failing to thrive?
inborn errors of metabolism T2DM
172
how do you calculate mid-parental height?
average of parents height
173
what is anthropometry?
in nutritional assessment - includes weight, height mid-upper arm circumference and skinfold thickness
174
what is Hirschprungs disease?
congenital condition where nerve cells of the myenteric (auerbach's) plexus are absent causing a lack of peristalsis of the large bowel
175
what innervation is responsible for motor supply to the bowel?
Auerbach plexus located between circular and longitudinal muscle layers of gut - responsible for peristalsis - in muscularis propria
176
what plexus is responsible for innervation of gastric absorption, secretion and blood flow as well as ion and water transport and sensory stimuli?
Meissner's plexus In submucosa
177
what is the key pathopysiology of hirschprungs disease?
absence of parasympathetic ganglion cells in the colon
178
what are 4 syndrome that can be associated with hirschprung's?
downs neurofibromatosis waardenburg syndrome multiple endocrine neoplasia type II
179
what are 5 presentations of hirschprungs disease?
delay in passing meconium chronic constipation since birth abdo pain and distention vomiting poor weight gain and failure to thrive
180
what is hirschsprung associated enterocolitis?
inflammation and obstruction of the intestines in 20% of neonates with hirschprungs presenting usually between 2-4 weeks with fever, abdo distension, diarrhoea (w/ blood usually) and features of sepsis
181
what is the management of hirschsprungs associated enterocolitis?
Abx fluid resuscitation decompression of obstruction
182
how is hirschsprungs disease diagnosed?
rectal biopsy + histology for absence of ganglionic cells
183
what is the management of hirschsprungs disease?
surgical resection of aganglionic bowel - usually anorectal pull-through
184
what age group does intussusception occur most commonly in?
6 months - 2 years more common in boys
185
what is intussusception?
when the bowel invaginates into itself usually causing bowel obstruction
186
what 5 conditions are risk factors for intussusception?
concurrant viral illness henoch-schonlein purpura CF intestinal polyps meckel diverticulum
187
what are 6 presentations of intussusception?
sever colicky abdo pain pale, lethargic unwell child Inconsolable crying drawing knees up and turning pale redcurrant jelly stool sausage shaped RUQ mass vomiting intestinal obstruction
188
what is the first line investigation for intussusception?
US abdo
189
what can be seen on abdo US in intussusception?
Target like mass
190
what is the treatment for intussusception?
1 - therapeutic enema (air insufflation) - contrast, water or air 2- surgery
191
what are 4 complications of intussusception?
obstruction gangrenous bowel perforation death
192
what are 8 causes of bowel obstruction in children?
meconium ileus hirschprungs oesophageal atresia duodenal atresia intussusception imperforate anus malrotation of the intestines + volvulus strangulated hernia
193
what can be seen on abdo xray in obstruction?
proximal dilated bowel loops distal collapsed bowel loops absence of air in rectum
194
what classes as bowel loop dilation in adults?
rule of 3s small bowl >3cm Large bowel >6cm Caecum >9 cm
195
when does biliary atresia present?
shortly after birth
196
what is the presentation of biliary atresia?
jaundice a few weeks after birth - CONJUGATED bilirubin Pale stools and dark urine irritability hepatomegaly Failure to thrive
197
what kind of bilirubin is high in biliary atresia?
conjugated bilirubin
198
what is the 1st line investigation for billiary atresia?
conjugated and unconjugated bilirubin
199
what is classed as persistent jaundice in babies?
>14 days term babies >21 days premies
200
what is the management of billiary atresia?
surgery - kasai portoenterostomy Ursoseoxycholic acid - given post op to promote bile flow often require full liver transplant
201
what surgery is used to correct biliary atresia?
Kasai portoenterostomy
202
what are 3 complications of biliary atresia?
Cholangitis cirrhosis and liver failure nutritional deficiencies - unable to absorb fat soluble vitamins
203
what is henoch-schonlein purpura?
autoimmune small vessel vasculitis usually triggered by URTI which causes inflammation of capilaries leading to purpuric rash typically on lower legs, joint pain and GI symptoms. Can also cause kidney damage due to IgA deposits
204
what are the 4 classic features of Henoch-schonlein purpura?
purpuric rash on legs joint pain GI symptoms Renal involvement - IgA nephritis
205
what is the management of henoch-schonlein purpura?
Supportive - analgesia (NSAIDs unless renal injury), rest, hydration Steroids may be used to shorted duration moniter urine dip and BP
206
what are 4 complications of henoch-scholein purpura?
GI bleed or intussuscpetion AKI or CKD Scrotal swelling or orchitis Recurrence
207
what is an abdominal migraine?
severe central abdo pain >1 hour with normal examination may also have nausea, vom, anorexia, pallor, headache, photophobia, aura
208
what is the acute management of abdo migraine?
paracetamol ibuprofen sumatriptan
209
what is the prophylaxis of abdo migraine?
1 - pizotifen - serotonin agonist propanolol cyproheptadine - antihistamine flunarazine - Ca channel blocker
210
what is gastroschisis?
a birth defect causing abdominal organs to be located outside abdomen due to defect in abdominal wall Vaginal delivery may be attempted and neonates are taken to theatre within 4 hours of birth
211
what are 2 examination findings in diaphragmatic hernia?
apex beat displaced to r side of chest poor air entry in L chest concave abdomen
212
what is the management of diaphragmatic hernia?
NG tube suction to prevent intrathoracic bowel distention and surgical repair - high mortality due to underdeveloped lungs in utero
213
who are umbilical hernias more common in?
children of african descent Down's syndrome mucopolysaccharide storage disease
214
what age do umbilical hernias usually resolve by?
3 years
215
what are 2 classes of symptoms of cow milk protein allergy?
GI symptoms - bloating, wind, abdo pain, diarrhoea , vomiting Allergic symptoms - hives, angio-oedema, cough/wheeze, sneezing, watery eyes, eczema, anaphylaxis
216
what formula can be used in cow milk protein allergy?
1 - hydrolysed formulas 2 - elemental amino acid formulas
217
what is one thing that can be prescribed to the breast feeding mother of cows milk protein allergy babies?
Calcium supplements - as they will not be eating dairy and therefore need another source of calcium
218
what is the difference between cow milk intolerance and allergy?
intolerance has GI symptoms but no allergic symptoms unlike allergy
219
by what age do children usually grow out of cow's milk protein allergy?
3 years
220
what immunoglobulin causes rapid cows milk protein allergy?
IgE
221
what are choledochal cysts?
congenital cyst dilations of the billary tree more commonly affecting females which have a small risk of malignancy usually surgically excised
222
what is the classic triad of choledochal cysts?
abdo pain jaundice abdo mass
223
what is neonatal hepatitis syndrome?
idiopathic prolonged neonatal jaundice and hepatic inflammation which may cause low birth weight and faltering growth
224
what is the usual presentation of colic?
sudden inconsolable crying/screaming accompanied by drawing knees to chest and passing excessive gas. Typically occurs <3 months, often worse in the evening
225
when should colic be suspected?
Rule of 3s more than 3 hours a day 3 days a week for 3 weeks otherwise healthy baby
226
what 2 findings indicate pyelonephritis over uti?
temp >38 degrees loin pain/tenderness
227
what is the management for < 3 months with fever?
immediate IV Abx - cefotaxime+ amoxicillin + full septic screen
228
what a full septic screen in <3 months?
bloods - FBC, CRP, cultures, lactate urine dip CXR LP ? cap gas
229
when should children with UTIs get an USS within 6 weeks?
if first UTI <6 months recurrent UTIs atypical UTIs
230
what investigation can be done for defects in renal tissue and scars after UTI?
DMSA scan (dimercaptosuccinic acid) - injection of radioactive material using gamma camera done 4-6 months after infection
231
what investigation can be done to visualise urinary anatomy, vesicouteric reflux and urethral obstruction in children <6 months?
micturating cystourethrogram (MCUG) - catheterise child and inject contrast then take x rays
232
what is vesico-uteric reflux?
where there is a developmental abnormality of the vesicouteric junction where the ureters are displaced laterally and enter the bladder directly allowing urine to reflux back up ureters and to the kidney causing dilatation and increased risk of infection and scaring
233
what are the 5 grades of vesicoureteric reflux?
1 - reflux to ureter only 2 - reflux to renal pelvis on micturition 3 - mild/moderate dilation of ureter, renal pelvis, calyces 4 - dilation of renal pelvis and calyces with moderate ureteral tortuosity 5 - gross dilation of ureter, pelvis and calyces with ureteral tortuosity
234
what is the management of pyelonephritis in children?
Consider referral to paeds at any age and definitely <3 months 1 - Cefalexin PO 3mon to 11 years - 12.5 mg/kg BD 7-10 days 12-15 years - 500mg BD/TDS 7-10 days OR Co-Amox PO 3mon-5 years - 0.25ml/kg of 125/31 suspension TDS 7-10 days IVs - Co-amox, cefuroxime, ceftriaxone, gent, amikacin
235
what is the management of UTI in children <3 months?
Imediate paeds referral IV antibiotics (ceftriaxone) Full septic screen - blood cultures, bloods, lactate, ?LP
236
what is the management of UTI in children >3 months?
1 - Trimethoprin PO 3mon to 11 years - 4mg/Kg BD for 3 days 12-15 years - 200mg BD 3 days Nitrofurantoin PO 3 mon-11 years - 750 microg/Kg QDS for 3 days 12-15 years - 50mg QDS 3 days 2 - Nitrofurantoin Cefalexin PO 3mon-11 years - 12.5mg/kg BD for 3 days 12-15 years - 500mg BD 3 days
237
what is enuresis?
involuntary urination
238
by what age do children usually get control of day time bladder function?
2 years
239
by what age do children usually stop bed wetting?
3-4 years
240
what are 5 possible causes of primary nocurnal enuresis?
overactive bladder - frequent small volume urination prevents development of bladder capacity fluid intake prior to bed failure to wake psychological distress secondary causes - chronic constipation, UTI, learning disability, cerebral palsy
241
what is primary nocturnal enuresis?
wetting the bed having never stopped
242
at what age does nocturnal enuresis become pathogenic?
5 years
243
what are 5 causes of primary nocturnal enuresis?
overactive bladder fluid intake failure to wake psychological distress secondary causes
244
what is secodnary nocturnal enuresis?
wetting the bed having previously stopped for >6 months
245
what are 5 causes of secondary nocturnal enuresis?
UTI constipation T1DM psychosocial problems abuse
246
what is diurnal enuresis?
day time wetting self
247
what are 3 management options for nocturnal enuresis?
1 - Eneurisis alarm 2 - desmopressin (taken at bedtime) 3- oxybutinin - anticholinergic imipramine - tricyclic antidepressant
248
what age range is nephrotic syndrome most common in?
2-5 years
249
what is the classical triad of nephrotic syndrome?
low serum albumin high urine protein (3+ or >3 grams) oedema
250
what are 6 signs of nephrotic syndrome?
low serum albumin high urine protein oedema deranged lipid profile high BP hyper-coagulability
251
what is the most common cause of nephrotic syndrome in children?
minimal change disease
252
what are 4 secondary causes of nephrotic syndromes?
intrinsic kidney disease - focal segmental glomerulosclerosis, mebranoproliferative glomerulonephitis Henoch schonlein purpura diabetes infection - HIV, hepatitis, malaria
253
what can be seen on urinalysis in minimal change?
small molecular weight proteins and hyaline casts
254
what is the first line management of minimal change disease?
high dose corticosteroids - prednisolone
255
what is the management of steroid resistant nephrotic syndrome?
ACEi immunosupressants - cyclosporine, tacrolimus, rituximab
256
what is the general management of nephrotic syndromes (5)?
high dose steroids - pred low salt diet diuretics albumin infusion antibiotic prophylaxis
257
what are 5 complications of nephrotic syndromes?
hypovolaemia and low BP thrombosis infection - kidneys leak immunoglobulins acute or chronic renal failure relapse
258
what is the pathophysiology of nephrotic syndromes?
the basement membrane of the glomerulus becomes highly permeable to proteins allowing them to leak from the blood into the urine
259
what is the pathophysiology of nephritic syndromes?
inflammation of the nephrons
260
what are 3 consequences of nephritic syndromes?
reduction in kidney function haematuria proteinuria (less than nephrotic)
261
what are the two most common causes of nephritis in children?
post-streptococcal glomerulonephritis IgA nephropathy - HSP
262
what is the pathophysiology of post-streptococcal glomerulonephritis?
1-3 weeks after b-haemolytic strep (strep pyogenes) infection e.g tonsilitis immune complexes get stuck in the mesangium of the glomeruli and cause inflammation leading to AKI
263
what 2 investigations can be done to test for strep in post-strep glomerulonephritis?
positive throat swab anti-streptolysin antibody titres
264
what is the management of nephritis?
supportive mainly manage complications - IgA may need immunosupression
265
what is IgA nephropathy a complication of?
Henoch-schonlein purpura (IgA vasculitis)
266
what is the pathophysiology of IgA nephropathy?
IgA deposits in nephrons cause inflammation
267
what 2 things can be seen on biopsy in IgA nephropathy?
IgA deposits Glomerular mesangial proliferation
268
what age group is usually affected with IgA nephropathy?
teens and young adults
269
what usually triggers haemolytic uraemic syndrome?
shinga toxin (from e.coli 0157 or shingella)
270
what is the classical triad of haemolytic uraemic syndrome?
haemolytic anaemia AKI thrombocytopenia
271
what increases the risk of haemolytic uraemic syndrome?
use of antibiotics and loperamide to treat gastroenteritis caused by e.coli0157 or shingella
272
how long after onset of gastroenteritis do symptoms of haemolytic uraemic syndrome usually start?
5 days
273
what are 8 presentations of haemolytic uraemic syndrome?
reduced urine output haematuria or dark brown urine abdo pain and bloody diarrhoea (due to gastroenteritis) lethargy and irritablility confusion oedema HTN bruising jaundice - due to haemolysis
274
what is the management of haemolytic uraemic syndrome?
supportive - renal dialysis, antihypertensives, maintain fluid balance, blood transfusion
275
what is hypospadias?
condition where uretheral meatus is displaced to underside of penis (ventral)
276
what is epispadias?
condition where the urethral meatus is displaced to the top side of the penis
277
what is chordee?
where head of penis is bent downwards
278
what is the management of hypospadias?
referral to paediatric urologist - tell patients to not circumcise baby surgery may be performed at 3-12 months
279
what are 3 complications of hypospadias?
difficulty directing urination cosmetic and psychological concerns sexual dysfunction
280
what is phemosis?
pathological non-retration of foreskin
281
what condition is the most common cause of phimosis?
balantitis xerotica obliterans
282
what reduction in renal function classes as AKI?
<0.5 ml/Kg/hour over 6 hours
283
what category of cause is most common in childhood AKI?
pre-renal
284
what are the 2 most common causes of intra-renal failure in children?
haemolytic uraemic syndrome acute tubular necrosis
285
what is stage 1 ckd?
eGFR >90 ml/min per 1.73 m2
286
what is stage 2 ckd?
eGFR 60-89
287
what is stage 3 ckd?
eGFR 30-59
288
what is stage 4 eGFR?
eGFR 15-29
289
what is stage 5 ckd?
eGGR <15 ml/min per 1.73m2
290
what 8 presenting features of severe CKD?
anorexia and lethargy polydipsia and polyuria faltering growth bony deformities from renal rickets hypertension acute on chronic renal failure proteinuria normochomic normocytic anaemia
291
what type of hypersensitivity reaction in anaphylaxis?
type 1
292
what immunoglobulin causes anaphylaxis?
IgE
293
what is the pathophysiology of anaphylaxis?
igE stimulates mast cells to rapidly release histamine in mast cell degranulation
294
what is the key feature of anaphylaxis vs non-anaphylactic allergy?
anaphylaxis causes compromise of airway, breathing or circulation
295
what are the 4 allergic symptoms?
urticaria itching angio-oedema abdominal pain
296
what is the management of anaphylaxis?
ABCDE IM adrenaline Antihistamines - chlorphenamine or certirizine steroids - hydrocortisone
297
what can be a complication of anaphylaxis?
biphasic reaction - second anaphylactic reaction after treatment
298
what investigation can be done for anaphylaxis?
serum mast cell tryptase - within 6 hours of event
299
what are 5 reasons someone with a non-anaphylactic allergic reaction may get an epipen?
asthma requiring ICS poor access to medical tx adolescents - higher risk nut/sting allergies significant co-morbidities
300
what is the skin sensitisation theory of allergy?
there is a break in the infants skin that allows allergens from the environment to react with the immune system but there is no contact with the allergen through the GI tract
301
what is the classification system for hypersensitivity reactions?
cooms and gell
302
what is a type 1 hypersensitivity reaction?
IgE antibodies trigger mast cells and basophils to degranulate causing an immediate reaction - food allergy reactions
303
what is a type 2 hypersensitivity reaction?
IgG and IgM mediated activating the complement system leading to direct damage of local cells - haemolytic disease of newborn, transfusion reactions
304
what is a type 3 hypersensitivity reaction?
immune complexes accumulate and cause damage to local tissues - autoimmune conditions
305
what is a type 4 hypersensitivity reaction?
cell mediated caused by T lymphocytes which are inappropriately activated causing inflammation and damage to local tissues - organ transplant rejection, contact dermatitis
306
what are 3 investigations for allergies?
skin prick test RAST testing - measure of allergen specific IgE food challenge - gold standard
307
what hypersensitivity reaction is allergic rhinits?
type 1 IgE
308
what is the presentation of allergic rhinitis?
runny, bocked, itchy nose sneezing itchy, red, swollen eyes clear nasal discharge post nasal drip
309
what are 3 non-sedating antihistamines?
certirizine loratadine fexofenadine
310
what are 2 sedating anti-histamines?
chlorpenamine promethazine
311
what can be taken as prophylactic for allergic rhinits?
nasal corticosteroids - fluticasone, mometasone
312
what are 3 live vaccinations?
BCG MMR nasal flu vaccine
313
what can cafe-au-lait spots be a sign of?
neurofibromatosis type 1 (if have 6+)
314
what 3 things can port wine marks rarely be a sign of?
sturge-weber syndrome klippel trenaunay syndrome macrocephaly-capillary malformation
315
what are mongolial blue spots?
blue/black macular discolouration at base of spine and on buttocks or thighs - fade over first few years of life
316
what is the name of the red skin mark that can grow up until a child's first birthday?
haemangioma - strawberry naevus
317
what is osteogenesis imperfecta?
genetic condition also known as brittle bone disease caused by mutation in formation of collagen
318
what is the inheritance of osteogenesis imperfecta?
Autosomal dominant
319
what are 8 features associated with osteogenesis imperfecta?
hypermobility blue/grey sclera triangular face short stature deafness from early adulthood dental problems bone deformities joint and bone pain
320
what are blood results usually like in osteogenesis imperfecta?
normal - calcium, phosphate, parathyroid, ALP
321
what is the pathophysiology of rickets?
deficiency in vitamin D/calcium which results in defective bone mineralisation
322
what is the name of the genetic version of rickets?
hereditary hypophasphataemic rickets
323
what is the inheritance of hereditary hypophosphataemic rickets?
X-linked dominant
324
what are 8 presentations of rickets?
lethargy bone pain swollen wrists bone deformuty poor growth dental problems muscle weakness pathological or abnormal fractures
325
what are 5 bone deformities in rickets?
bowing of legs knock knees rachitic rosary - expanded ribs causing lumps on chest craniotabes - soft skull, delayed closing delayed teeth
326
what investigation is done for vitamin d deficiency?
serum 25-hydroxyvitamin D - <25nmol/L = deficiency
327
what are 5 investigations that can be done for rickets?
serum 25-hydroxyvitamin D serum calcium serum phosphate serum alkaline phosphatase parathyroid hormone
328
what is the most common cause of hip pain in children 3-10 years?
transient synovitis
329
what is transient synovitis often associated with?
preceeding viral upper resp tract infection low grade fever
330
what criteria can be used to distinguish between transient synovitis and septic arthritis in children?
Kocher criteria
331
what is the Kocher criteria?
For septic arthritis in children Fever >38.5 Non-weight bearing Raised ESR Raised WCC
332
what age is septic arthritis most common in?
<4 years
333
what is perthes disease?
disruption of blood flow to femoral head causing avascular necrosis of the epiphysis of the femur
334
what age group does perthes disease occur most commonly in?
Occurs 4-12 years age group most commonly 5-8 years More prevalent in boys
335
what is the presentation of perthe's disease?
slow onset of Pain inn hip/groin Limp Restricted hip movements Referred pain to knee
336
what investigation is used to confirm absence of perthe's disease in normal x-ray?
Technertium bone scan OR MRI
337
what staging can be used for perthe's disease?
Catterall staging
338
what are 2 complications of perthe's disease?
Osteoarthritis premature fusion of growth plates
339
what is the management of perthe's disease?
Bed rest traction crutches analgesia physio regular x-rays Surgery may be required if present at older age or greater stage of disease
340
what is slipped upper femoral eiphysis? (SUFE)
when head of femur displaces along growth plate
341
who is SUFE more common in?
boys aged 8-15 obese children May be history of mild trauma
342
how do people with SUFE prefer to hold their hip?
in external rotation and have limited movement of hip
343
what is the 1st line investigation for SUFE?
X-ray - AP and Lateral (frog leg) views
344
what is the management of SUFE?
Avoid weight bearing surgery to correct femoral head position - internal fixation
345
what are 4 complications of SUFE?
Osteoarthritis Avascular necrosis of the femoral head Chondrolysis Leg length discrepancy
346
what is developmental dysplasia of the hip?
structural abnormality n developmental of foetal bones leading to instability of the hips and tendency for subluxation or dislocation
347
what are 7 risk factors for developmental dysplasia of the hip?
FHx 1st degree relative breech presentation > 36 weeks or at birth Multiple pregnancy Female oligohydramnios prematurity macrosomnia (>5kg)
348
what are 6 findings that may suggest developmental dysplasia of the hip on neonatal examination?
different leg lengths restricted hip abduction on one side significant bilateral restriction in hip abduction difference in knee level with flexed hips clunking of hips on special tests asymmetrical skin folds
349
what are two special test for developmental dysplasia of the hio?
ortolani test barlow test
350
what infants require an US for developmental dysplasia of the hip? 3
1st degree relative Hx of hip problem in early life Breech at or after 36 weeks Multiple pregnancy
351
what is the ortolani test?
for developmental dysplasia of the hip hips and knees flexed, hips gently abducted and pushed from the back to see if they will dislocate anteriorly
352
what is barlow test?
for developmental dysplasia of the hip hips and knees flexed and gentle pressure put on knees through femur to see if hips will dislocate posteriorly
353
what investigation can be done for developmetal dysplasia of the hips?
US hip
354
what is the 1st line investigation for developmental dysplasia of the hip if presenting >4.5 months old?
X-ray hip
355
what is the management for developmental dysplasia of the hips?
Pavlik harness (<6 months) for 6-8 weeks surgery if harness fails or >6 months old and then hip spica cast
356
what are 5 complications of developmental dysplasia of the hip?
Pain Hip instability early onset hip osteoarthritis femoral nerve palsy avascular necrosis of the femoral head
357
what are the 5 subtypes of juvenile idiopathic arthritis?
systemic JIA polyarticular JIA Oligoarticular JIa enthesitis related arthritis juvenile psoriatic arthritis
358
what is systemic juvenile idiopathic arthritis also known as?
Still's disease
359
what are 8 manifestations of systemic JIA?
subtle salmon pink rash high swinging fevers enlarged lymph nodes weight loss joint inflammation and pain splenomegally muscle pain pleuritis and pericarditis >6 weeks
360
what is seen on blood tests in systemic JIA?
negative - antinuclear antibodies, RF Raised CRP, ESR, platelets, ferritin
361
what is are 2 key complication of systemic JIA?
macrophage activation syndrome - severe inflammatory responce causing DIC, anaemia, thrombocytopenia, bleeding, non-blanching rash chronic anterior uveitis
362
what are 4 key non-infective causes of fever in children > 5 days?
kawasaki disease still disease (systemic JIA) rheumatic fever leukaemia
363
what is polyarticular JIA?
idiopathic inflammatory arthritis in 5+ joints - equivalent of RhA in adults though most children are seronegative
364
what is oligoarticular JIA?
monoarthritis affecting <4 joints, classically associated with anterior uveitis. most common in gils under 6
365
what is enthesitis related arthritis?
paediatric seronegative spondyloarthropathies. inflammatory arthritis + enthesitis (inflammation of tendon insertion points) usually HLA-B27 +ve
366
what are 5 signs of juvenile psoriatic arthritis?
places of psoriasis nail piitting onycholysis (seperation of nail bed) dactylitis enthesitis
367
what is the management of juvenile idiopathic arthritis?
NSAIDs steroids DMARDs - methotrexate Biologics - TNF inhibitors, infliximab etc
368
what age group does kawasaki's usually affect?
<5 years
369
what is a key complication of kawasaki disease?
coronary artery aneurysm
370
what is the pathophysiology of kawasaki disease?
systemic medium sized vessel vasculitis?
371
what are 7 key features of kawasaki?
high (>39) fever >5 days widespread erythematous maculopapular rash on trunk Oedema of hands and feed preceding desquamation Strawberry tongue and cracked lips cervical lymphadenopathy bilateral conjunctivitis arthritis
372
what are 5 investigations for kawasaki?
FBC - anaemia, leukocytosis, thrombocytosis LFTS - hypoalbuminaemia, elevated enzymes raised inflammatory markers (particularly esr) Urinaralysis - wt cells without infection ECHO
373
what happens in the acute phase of kawasakis?
most unwell for 1-2 weeks Fever, rash, lymphadenopathy
374
what happens in the subacute phase of kawasakis?
weeks 2-4 acute symptoms settle desquamation, strawberry tongue and arthralgia occur - there is risk of coronary artery aneurysms forming
375
what happens in the covalescent stage of kawasakis?
week 2-4 remaining symptoms settle
376
what is the management of kawasaki disease?
high dose aspirin - reduce risk of throbosis IV Ig to reduce risk of coronary artery aneurysm
377
what is Reye's syndrome
a preceeding viral infection usually treated with ASPIRIN followed by acute encephalopathy and hepatic dysfunction in children and young people
378
what are 4 complications of Kawasaki disease?
coronary artery aneurysm Myocarditis pericarditis Reye's syndrome
379
what follow up is needed with kawasakis?
ECG and ECHO
380
what cells produce surfactant?
type II pneumocytes
381
when does surfactant start to be produced?
between 24-34 weeks gestation
382
what is required to keep the ductus arteriosus open?
prostaglandins
383
what can extended hypoxia lead to during birth?
hypoxic-ischaemic encephalopathy => cerebral palsy
384
what are 3 issues of neonatal resuscitation?
large SA to weight ratio - cold born wet and loose heat rapidly meconium aspiration
385
what are 5 principles of neonatal resuscitation?
warm baby calculate APGAR score stimulate breathing inflation breaths chest compressions
386
when should you calculate the APGAR score?
1, 5 and 10 minutes during resus
387
how do you stimulate breathing in neonatal resussitation?
dry vigorously with towel place baby head in neutral check for airway obstruction and consider aspiration if gasping or unable to breathe
388
how should inflation breaths be given in neonates?
2 cycles of 5 inflation breaths if no response 30s of ventilation breaths can be used then chest compressions coordinated with ventilation breaths
389
what should be used for inflation breaths in preterm vs term babies?
preterm - air and O2 term - just air
390
when should you start chest compressions in a neonate?
if HR <60 bpm despite resus and inflation breaths
391
what is the APGAR score?
for neonatal resus Appearance (skin colour) Pulse Grimmace (to stimulation) Activity (tone) respiration
392
what is the scoring for appearance in APGAR?
blue/pale centrally = 0 blue extremities = 1 pink = 2
393
what is the scoring for pulse in APGAR?
Absent = 0 <100 = 1 >100 = 2
394
what is the scoring for grimmace in APGAR?
no response = 0 little response = 1 good response = 2
395
what is the scoring for activity in APGAR?
floppy = 0 flexed arms and legs =1 active = 2
396
what is the scoring for respiration in APGAR?
absent =0 slow/irregular = 1 strong/crying = 2
397
how long should delayed cord clamping be?
1 minute
398
under what gestation can be affected by respiratory distress syndrome?
< 32 weeks
399
what xray changes are seen in respiratory distress syndrome?
ground glass appearance
400
what are 4 types of ventilatory support that may be needed by premature neonates?
intubation and ventilation endotracheal surfactant CPAP supplementary oxygen
401
what are 6 short term complications of respiratory distress syndrome?
penumothorax infection apnoea intraventricular haemorrhage pulmonary haemorrhage necrotising enterocolitis
402
what are 3 long term complications of respiratory distress syndrome?
chronic lung disease of prematurity retinopathy of prematurity neurological, hearing or visual impairment
403
what is the management of neonatal respiratory distress syndrome?
Antenatal maternal glucocorticoids as prophylaxis Raised ambient Ox - aim 92-95% sats Surfactant therapy mechanical ventilation
404
what is transient tachypnoea of the newborn?
benign self limiting condition caused by delay in reabsorption of lung liquid after birth Usually self resolves within 1st day of life
405
what is the 1st line investigation for transient tachypnoea of the newborn?
CXR - fluid in horizontal fissure
406
what are 3 risk factors for transient tachypnoea of the newborn?
prematurity gestational diabetes c-section
407
when can physiological jaundice be present?
from 2-10 days of age
408
what are 8 causes of increased production of bilirubin in neonates?
haemolytic disease of newborn ABO incompatibility Haemorrhage intraventricular haemorrhage cephalo-haemorrhage polycythaemia sepsis and DIC G6PD deficiency
409
what are 6 causes of decreased bilirubin clearance in neonates?
prematurity breast milk jaundice neonatal cholestasis extrahepatic biliary atresia endocrine disorders gilbert syndrome
410
when is jaundice always pathological?
in first 24 hours of life can be sign of sepsis
411
what is kernicterus?
brain damage due to high unconjugated bilirubin
412
what causes haemolytic disease of the newborn?
rhesus incompatability
413
what is prolonged neonatal jaundice?
14+ days in full term babies 21+ days in premature babies
414
what test is done for autoimmune haemolysis?
direct coombs test
415
what is used to monitor neonatal jaundice?
treatment threshold charts
416
what is the usual treatment of neonatal jaundice?
phototherapy with blue light (450nm)
417
what are 3 complications of kernictus?
cerebral palsy learning disability deafness
418
what is the medical management of supraventricular tachycardia?
adenosine - after valsalva manoeuvres
419
what is necrotising enterocolitis?
disorder affecting premature neonates where part of bowel becomes necrotic and can lead to perforation
420
what are 5 risk factors for necrotising enterocilitis?
very low birth weight or v premature formula feeds resp distress and assisted ventilation sepsis patent ductus arteriosus and congenital heart disease
421
what are 6 features of necrotising enterocolitis?
intolerance to feeds vomiting green bile distended tender abdomen blood in stool absent bowel sounds generally unwell
422
what investigation is good for diagnosing necrotising enterocolitis?
abdo Xray - supine, lateral, lateral decubitus
423
what can be seen on xray with necrotising enterocolitis?
dilated bowel loops bowel wall oedema pneumatosis intestinalis - gas in bowel wall pneumoperitoneum - free gas in peritoneum gas in portal veins
424
what is the management of necrotising enterocolitis?
nil by mouth iv fluids total parenteral nutrition antibiotics NG tube - to drain fluid and gas SURGERY
425
what are 8 complications of necrotising enterocolitis?
perforation and peritonitis sepsis death strictures abcess formation recurrence long term stoma short bowel syndrome
426
what are the torch congenital infections?
Toxoplasmosis rubella cytomegalavirus herpes simplex HIV
427
at what gestation is the risk of congenital rubella highest?
< 3 months
428
what are 3 features of congenital rubella?
Classic triad of - deafness, blindness (congenital cataracts) and congenital heart disease also learning disability growth retardation hepatosplenomegaly purpuric skin lesions salt and pepper chorioretinitis microphthalmia cerebral palsy
429
can pregnant women be given the MMR vaccine?
NO should give to non-immune mothers postnatally
430
what are 6 features of congenital cytomegalovirus?
foetal growth restriction microcephaly hearing loss vision loss learning disability seizures
431
what is the classic triad of congenital toxoplasmosis gondii?
intracranial calcification hydrocephalus chorioretinitis (type of posterior uveitis)
432
what are 6 manifestations of neonatal herpes?
external herpes lesions liver involvement encephalitis sezures, tremmors, lethargy, irritability
433
what are 5 complications of chicken pox in pregnancy?
foetal varicellar zoster syndrome pneumonitis hepatitis encephalitis severe neonatal infection
434
what are 6 manifestations of congenital varicella syndrome?
foetal growth restriction microcephaly hydrocephalus, learnign disability scars and skin changes limb hypoplasia cataracts and chorioretinitis
435
what is given to a baby immediatly after birth?
vitamin K IM injection
436
what 9 conditions are tested for in the blood spot test?
sickle cell CF congenital hypothyroidism phenylketonuria medium chain acy-COA dehydrogenase deficiency maple syrup urine disease isovaleric acidaemia glutaric aciduria type 1 homocystin
437
at what age does the heel prick test happen?
5 days old
438
how long does the heel prick take to come back?
6-8 weeks
439
when is the NIPE examination done?
<72 hours of birth
440
when is the NIPE repeated?
6-8 weeks by GP
441
when does the ductus arteriosus close?
1-3 days
442
how small should the difference in pre-ductal and post-ductal O2 saturations be in the NIPE?
<2% pre-ductal - R hand post ductal - feet
443
what is the name of oedema of the scalp outside the periosteum due to trauma in birth which crosses suture lines?
caput succedaneum
444
what is the name of a collection of blood between the skull and periosteum due to trauma in delivery which does not cross suture lines?
cephalohaematoma
445
what is erbs palsy?
damage to C5/6 brachial plexus leading to weakness in shoulder abduction and external rotation, arm flexion and finger extension may have a 'waiters tip appearance' - internal rotation of shoulder with elbow extension
446
what are 5 common organisms in neonatal sepsis?
GBS - most common E. Coli - most common Listeria Klebsiella staph aureus
447
what is early onset neonatal sepsis?
<72 hours
448
what is late onset neonatal sepsis?
Onset days 7-28
449
what are 6 risk factors for neonatal sepsis?
vaginal GBS colonisation GBS sepsis in previous baby Maternal sepsis, chorioamniotitis or fever prematurity premature ROM prolonged rupture of membranes low birth weight <2.5kg
450
what are 6 red flags for neonatal sepsis?
confirmed/suspected sepsis in mother signs of sock seizures term baby needing mechanical ventilation resp distress starting 4+ hours after burth presumed sepsis in other multiple
451
what are 10 clinical features of neonatal sepsis?
fever reduced tone and activity poor feeding resp distress or apnoea vomiting tachy or brady hypoxia jaundice <24 hours seizures hypoglycaemia
452
what is the nice treatment for neonatal sepsis?
monitor if 1 risk factor/clinical feature start antibiotics within one hour if 2 risk factors/clinical features or 1 red flag
453
what antibiotic should be given in neonatal sepsis?
benzylpenicillin PLUS gentamycin cefotaxime if lower risk
454
what is the ongoing management of neonatal sepsis?
check CRP at 24 hours and blood cultures at 36 hours consider stopping Abx if blood cultures negative and CRP <10 at day 2
455
what counts as extreme preterm?
<28 weeks
456
what counts as very preterm?
28-32 weeks
457
what counts as moderate/late preterm?
32-37 weeks
458
what are 10 early complications of prematurity?
respiratory distress syndrome hypothermia hypoglycaemia poor feeding apnoea and bradycardia neonatal jaundice intraventricular haemorrhage retinopathy of prematurity necrotising enterocolitis immature immune system and infection
459
what are 5 long term complications of prematurity?
chronic lung disease of prematurity learnign an dbehvioural difficulties susceptibility to infections - particularly resp hearing and visual impairement cerebral palsy
460
what is apnoea?
stopping breathing >20 seconds or shorter periods with oxygen desaturation or bradycardia
461
what is the causes of apnoea in neonates?
due to immaturity of the autonomic nervous system
462
what is the management of neonatal apnoea?
tactile stimulation IV caffeine
463
what is the pathophysiology of retinopathy of prematurity?
retinal blood vessel formation is stimulated by hypoxia which is the normal condition of the retina during pregnancy so with early exposure to oxygen in prematurity this can stop retinal blood vessel development and cause retinal detachment
464
who should screening for retinopathy of prematurity be offered to?
<32 weeks gestation <1.5kg
465
what is the management of retinopathy of prematurity?
transpupillary laser photocoagulation cyotherapy injections of intravitreal VEGF inhibitors Surgery if retinal detachment
466
how is a diagnosis of meconium aspiration confirmed?
CXR
467
what are 3 foetal risk factors for meconium aspiration?
gestational age >42 weeks foetal distress APGAR score <7
468
what ae 5 maternal risk factors for meconium aspiration?
HTN pr-eclampsia chorioamnionitis smoking substance abuse
469
what is the management for suspected HSV encephalitis?
aciclovir
470
How do you get infected with listeria?
consumption of dairy products, raw vegetables, meats and refrigerated foods passed from mother to baby placentally
471
what are clinical manifestations of neonatal listeriosis?
abortion prematurity still birth neonatal sepsis
472
how can listeria infection be diagnosed prenatally and postnatally?
culture or PCR of blood, cervix or amniotic fluid of mother prenatally culture/PCR of blood, CSF, gastric aspirate, meconium of neonate
473
what is the management of neonatal listeriosis?
Ampicillin +/- gentamicin for 21 days
474
what is maternal hyperthyroidism associated with?
foetal tachycardia small for gestational age prematurity still birth congenital malformations
475
what is maternal hypothyroidism associated with?
lower IQ and impaired psychomotor development
476
what are 6 risk factors for neonatal hypoglycaemia?
IUGR preterm Maternal diabetes hypothermia Neonatal sepsis Inborn errors of metabolism Nestidiolastosis Beckwith-wiedemann syndrome
477
what are 8 symptoms of neonatal hypoglycaemia?
jitters irritability tachypnoea lethargy weak cry drowsiness hypotonia seizures apnoea and hypothermia
478
what counts as hypoglycaemia in neonates?
<2.6 mmol/L
479
what is the management of asymptomatic hypoglycaemia in neonates?
Encourage normal feeding monitor BMs
480
what are 3 preventative measures for neonatal hypoglycaemia?
Keep warm skin to skin Early feeding - within 1 hour of birth
481
what is the management of symptomatic hypoglycaemia or very low BMs in neonates?
admission to neonatal unit IV 10% dextrose
482
what is the pathophysiology of cleft lip and palate?
lip - failure of fusion of the frontonasal and maxillary processes Palate - failure of fusion of palatine processes and nasal septum
483
when is a cleft lip and palate usually fixed?
lip - 3 months palate - 6 months - 1 year
484
what are 2 USS signs in pregnancy of oesophageal atresia?
polyhydramnios no stomach bubble
485
what is a clinical sign of oesophageal atresia in neonates?
persistant salivation and drooling
486
what is the name of a group of midline congenital defects like oesophageal atresia?
VACTERL association Vertebral anorectal cardiac tracheo-oesophageal renal limb anomalies
487
what is the management of oesophageal atresia?
continuous suction of secretions to avoid aspiration until surgery is available
488
what is an exomphalos?
where abdominal contents protrude through umbilical ring but are covered by transparent sac formed by amniotic membrane and peritoneum
489
what are 3 conditions associated with exomphalous?
Beckwith-Wiedemann syndrome Down's syndrome cardiac and kidney malformations
490
what is the management of exomphalos?
Caesarean section staged repair until sac is able to fit inside abdomen of baby
491
what congenital condition is duodenal atresia associated with?
downs syndrome
492
what is an x-ray/USS sign of duodenal atresia?
'double bubble' of of distention of stomach and duodenal cap with absence of air distally
493
what are 3 obstetric complications of diabetes in pregnancy?
polyhydramnios preeclampsia early and late foetal loss
494
what are 3 foetal complications of diabetes in pregnancy?
congenital malformations IUGR macrosomia
495
what are 4 neonatal complications of diabetes in pregnancy?
neonatal hypoglycaemia respiratory distress syndrome hypertrophic cardiomyopathy polycythaemia
496
what is scoliosis?
lateral curvature of frontal plane of spine. In severe cases can lead to cardiorespiratory failure from curvature of chest
497
what is torticollis?
Head twisting to the side also called wry neck. The most common cause in infants is a sternomastoid tumour.
498
what is the incubation period for mumps?
14-25 days
499
what pathogen causes mumps?
RNA paramyxovirus
500
what is the presentation of mumps?
prodromal fever, muscle aches, lethargy, reduced appetite, headache and dry mouth for a few days followed by painful parotid swelling usually self limiting after around a week
501
what are 4 complications of mumps?
pancreatitis orchitis meningitis sensorineural hearing loss
502
How is mumps diagnosed?
salivary PCR salivary or blood antibodies
503
what is the management for mumps?
supportive - self limiting infection
504
what is the rash and its spread in chicken pox?
widespread itchy erythematous raised vesicular blistering lesions - starts as macules then papules then vesicles Starts on trunk or face and spreads outwards over 2-5 days the crusts after 5 days
505
what are 5 complications of chicken pox?
bacterial superinfection (necrotising faciitis) dehydration conjunctival lesions pneumonia encephalitis (presents with ataxia)
506
what is Ramsay hunt syndrome?
Unilateral weakness or paralysis of facial nerve due to reactivation of varicella zoster (type of shingles)
507
what medication may increase risk of secondary bacterial infection in chickenpox?
NSAIDs
508
How long can it take someone to become symptomatic after exposure to chicken pox?
10 days - 3 weeks
509
what is the management of neonatal varicella zoster infection?
varicella zoster Ig Aciclovir
510
what is the management of chickenpox?
Aciclovir - if immunocompromised, >14 years and presenting within 24h, neonates Calamaine lotion and chlorphenamine for symptoms
511
what is the presentation of measles?
fever, coryza and conjunctivitis followed 2 days later by greyish white spots on the buccal mucosa - KOPLIK SPOTS followed by rash 3-5 days post fever, classically starting behind ears self limiting after 7-10 days
512
what sign on examination is pathognomonic for measles?
Koplik spots - grey/white spots on buccal mucosa that appear 2 days after fever
513
what is the rash like in measles and how does it spread?
starts on face (classically behind ears) 3-5 days after fever and then spreads to rest of body erythematous macular rash with flat lesions
514
what is the incubation period of measles?
10-12 days
515
what are 8 complications of measles?
pneumonia - most common cause of death diarrhoea dehydration encephalitis - 1/2 weeks later meningitis hearing loss and otitis media - most common vision loss death subacute sclerosing panencephalitis - CNS demyelination 5-10 years post measles exposure
516
what can be given prophylactically after measles exposure in an unvaccinated person?
MMR vacine
517
what is the school exclusion criteria for measles?
4 days after development of rash
518
what pathogen causes scarlet fever?
exotoxins from group A haemolytic strep (strep pyogenes)
519
what is the rash in scarlet fever and how does it spread?
red-pink blotchy macular fine pinhead rash with rough SANDPAPER skin Starts on trunk and spreads outwards spearing palms and soles may also have flushed cheeks
520
what are 7 features of scarlet fever?
sandpaper rash fever - 24-48h lethargy. headache, nausea flushed face sore throat Strawberry tongue cervical lymphadenopathy
521
what is the management of scarlet fever?
1 - phenoxymethylpenicillin (penicillin V) for 10 days 2 - azithromycin in PENICILLIN ALLERGY - 5 days
522
what is the school exclusion for scarlet fever?
Return 24h after commencing Abx if feel well enough to do so
523
what are 4 complications of scarlet fever?
post-streptococcal glomerulonephritis acute rheumatic fever - typically 20 days after infection Otitis media - most common Invasive complications - bacteraemia, meningitis
524
how long is the incubation time for rubella?
2 weeks
525
what is the rash in rubella and how does it spread?
Erythematous macular rash (milder than measles) starts on face and spreads to body lasts 3 days associated with mild fever, joint pain, sore throat, lymphadenopathy
526
what are 4 rare complications of rubella?
thrombocytopenia encephalitis arthritis myocarditis
527
what are 5 presenting features of rubella?
erythematous macular rash mild fever joint pain sore throat lymphadenopathy
528
what investigation can be done for rubella?
oral fluid sample
529
what is the school exclusion criteria for rubella?
5 days from onset of rash
530
what pathogen causes slapped cheek syndrome?
Parvovirus b19
531
what is the presentation of parvovirus B19?
fever, coryza and non-specific viral symptoms followed 2-5 days later by diffuse bright red rash on both cheeks (as though slapped) a few days later a reticular mildly erythematous affecting trunk and limbs - may be raised and itchy
532
what are 4 complications of parvovirus?
aplastic anaemia - sickle cell/thalssaemia/spherocytosis encephalitis or meningitis pregnancy complications - foetal death due to hydrops fetalis rare - hepatitis, myocarditis, nephritis
533
what pathogen causes roseola infantum?
HHV-6/7
534
what is the presentation of roseola infantum?
presents 1-2 weeks after high fever for 3-5 days which the settles and is followed by a rash
535
what is the rash like in roseola infantum?
mild erythematous macular rash on arms, legs, trunk and face that isn't itchy
536
what are 3 complications of roseola infantum?
febrile convulsions rare - myocarditis, thrombocytopenua, GBS
537
what pathogen causes whooping cough?
bordetella pertussis - gram neg
538
what is the presentation of whooping cough?
mild coryzal symptoms and low grade fever, dry cough followed by more severe coughing fits after 1 week so much so people may vomit, faint or have a pneumothorax infants may present with apnoea spells
539
what are 2 ways to diagnose whooping cough?
nasopharangeal PCR or culture anti-pertussis toxin IgG if cough present >2 weeks
540
what is the management of whooping cough?
1 - macrolides - azithromycin, erythromycin, clarithromycin within first 21 days 2 - Co-trimoxazole
541
what is a complication of whooping cough?
bronchiectasis
542
what can be given to vulnerable contacts of people with whooping cough?
prophylactic antibiotics
543
when are infants vaccinated against pertussis?
2, 3, 4 months 3-5 years
544
when are pregnant women offered the pertussis vacciantion?
16-32 weeks
545
what bacteria causes diphtheria?
corynebacterium diphtheriae
546
what are 5 features of diphtheria?
sore throat + difficulty swallowing fever lymphadenopathy - bull neck SOB pseudomembrane on tonsils and mucosa of pharynx larynx and nose
547
what is one cardiac complication of diphtheria?
heart block
548
what is the management of diphtheria?
1 - diphtheria antitoxin Abx - azithromycin, erythromycin, clarithromycin, IM penicillin
549
what is the pathogenic cause of scalded skin syndrome?
staph aureus epidermolytic toxins
550
what age group is usually affected by scalded skin syndrome?
<5 years
551
what sign is positive in scalded skin syndrome?
Nikolysky sign - gentle rubbing of skin causes it to peel away
552
what is a major acute complication of polio?
acute flaccid paralysis
553
what is a possible long term complication of polio?
post-poliomyelitis syndrome - weakness and fatigue of muscle groups affected in acute illness
554
what is the main cause of viral encephalitis in neonates?
HSV-2 - genital herpes
555
what is the most common cause of viral encephalitis?
HSV-1 - from cold sores
556
what is the treatment for encephalitis caused by CMV?
ganciclovir
557
what are 4 complications of encephalitis?
change in personality, memory or cognition leaning disability headache movement disorders
558
what is the most common cause of toxic shock syndrome?
group A strep (pyogenese) exotoxins or s.aureus toxins
559
what are 5 risk factors for toxic shock?
Extended tampon use Post partum infection surgical procedures - breast reconstruction, hysterectomy, lipo Other infections burns retained foreign body
560
what are 6 presenting features of toxic shock?
severe diffuse or localised pain in an extremity fever hypotension diffuse, scarlatina-like red rash, may desquamate muscle weakness multiorgan failure - 3+ organs
561
what is the management of strep toxic shock?
Removal of infection focus IV fluids clindamycin + benzylpenicillin or vancomycin 7-14 days
562
what is the management for staph toxic shock?
clindamycin + oxacillin or vancomycin
563
what are 4 complications of toxic shock?
bacteraemia acute resp distress DIC renal failure
564
what is the management for oral candidaiasis?
miconazole gel nystatin suspension fluconazole tablets
565
what does cutaneous candida rash look like?
sore and itchy pustular or papular erythematous rash with an irregular edge and SATELLITE lesions often in flexural areas
566
what is the management of nappy rash?
1 - topical hydrocortisone 1% if inflamed topical imidazoles (clotrimazole), oral antibiotics if severely inflammed
567
what causes hand foot and mouth disease?
coxsackie A16 virus and enterovirus 71
568
what is the incubation period for hand foot and mouth?
3-5 days
569
what is the typical history for hand foot and mouth disease?
resp illness + fever 1-2 days later - mouth ulcers then red painful possibly itchy blistering spots across body notably on hands, feet and in mouth
570
what is the management for hand foot and mouth?
supportive - will resolve in 7-10 days without treatment does not need school exclusion
571
what are 3 complications of hand foot and mouth disease?
dehydration bacterial superinfection encephalitis
572
what kind of bacteria in Neisseria meningitidis?
gram negative diplococci
573
what is the most common cause of meningitis in neonates?
group B strep
574
what are 5 non-specific signs of meningitis in neonates?
hypotonia poor feeding lethargy hypothermia bulging fontanelle
575
what age group do febrile convulsions occur in?
ages 6 months - 5 years
576
what are simple febrile convulsions?
one generalised tonic clonic <15 minutes Complete recovery within an hour No recurrence within 24 hours
577
what is a complex febrile seizure?
15-30 minutes Focal seizure Repeat seizures in 24 hours
578
when should parents phone an ambulance in febrile seizure?
at 5 minutes
579
do antipyretics reduce risk of febrile seizure?
NO!
580
what rescue medication can be given in those with recurrent febrile seizures?
Bucal midazolam Rectal diazepam
581
what are 5 possible causes of global developmental delay?
downs fragile X foetal alcohol syndrome rett syndrome Metabolic disorders
582
what are 8 manifestations of foetal alcohol syndrome?
microcephaly thin upper lip smooth flat philtrum short palpebral fissure Cardiac malformations learning difficulties behavioural difficulties hearing and vision problems cerebral palsy
583
what are 5 possible causes of gross motor delay?
cerebral palsy ataxia myopathy spina bifida visual impairement
584
what are 5 possible causes of fine motor delay?
dyspraxia cerebral palsy muscular dystrophy visual impairment congenital ataxia
585
what are 6 possible causes of language delay?
social circumstance hearing impairement learning disability neglect autism cerebral palsy
586
what are 3 possible causes of social delay?
emotional and social neglect parenting issues autism
587
what are the 4 areas of development?
gross motor fine motor and vision language and hearing personal and social
588
what is the gross motor development of a 3/4 month old?
start supporting head and keep in line with body
589
what is the gross motor development at 6 months?
Maintain sitting position usually supported as unbalanced Rolls front to back
590
at what age do babies usually sit unsupported at at what age should they be referred?
7-8 mon refer if not by 12 months
591
what is the gross motor development at 15 months?
walk unaided
592
what is the gross motor development at 9 months?
sit unsupported start crawling maintain standing and bouncing position wile supported
593
what is the gross motor development at 12 months?
standing and cruising
594
what is the gross motor developement at 18 months?
squat and pick things up refer if not walking unsupported by 18 months
595
what is the gross motor development at 2 years?
run and kick ball
596
what is the gross motor development at 3 years?
climb stairs one foot at time stand on one leg ride tricycle
597
what is the gross motor development of a 4 year old?
hop climb stairs normally
598
what are 8 red flags of developemental milestones?
lost development unable to hold object 5 months not sitting unsupported at 12 months not standing independently 18 months not walking 2 years not running 2.5 years no words 18 months no interest in others 18 months
599
what is social development at 6 weeks?
smiles
600
what is social development at 3 months?
communicates pleasure
601
what is social development at 6 months?
curious and engages with people
602
what is social development at 9 months?
apprehensive around strangers
603
what is social development at 12 months
engages with others pointing and handing objects waves bye claps
604
what is social development at 2 years?
interest in others beyond parents parallel play
605
what is social development by 3 years?
play with others bowel control
606
what is social development by 4 years?
has best friend dry at night dresses self imaginative play
607
what is language development at 3 months?
cooing turns towards sounds
608
what is language development at 9 months?
babbling - mama, dada
609
what is language development at 12 months?
say 1 word in context follows simple instructions knows and responds to own name
610
what is language development at 18 months?
5-10 words
611
what is language development at 2 years?
combines 2 words
612
what is language development at 3 years?
basic 3-5 word sentences identifies colours asks what and who questions
613
what is language development at 4 years?
tells stories asks why, when and how questions
614
what is fine motor development at 8 weeks?
fixes and attempts to follow recognises faces
615
what is fine motor development at 6 months?
palmar grasp pass objects hand to hand
616
what is fine motor development at 9 months?
scissor grasp between thumb and forefinger points with finger
617
what is fine motor development at 12 months?
pincer grasp with tip of thumb and forefinger scribbles randomly bangs toys
618
what is fine motor development at 14-18 months?
clumsily use cutlery tower of 3 blocks
619
what is the first line management for absence seizures?
ethosuximide
620
when do infantile spasms usually start?
4-8 months more common in males
621
what are 3 features of infantile spasms?
Attacks - flexion of head and trunk followed by extension of arms Lasts 1-2 seconds but is repeated up to 50 times Progressive mental handicap
622
what is the prognosis for infantile spasms?
1/3rd die by 25 1/3rd keep seizures 1/3rd seizure free
623
what is seen on EEG in infantile spasm?
Hypsarrhythmia
624
what are 2 treatment options for infantile spasms?
1 - Vigabatrin ACTH - adrenocorticotropic hormone
625
what is klinefelter syndrome?
when a male has a additional X chromosomes making them 47XXY
626
what is the presentation of Klinefelters syndrome? (8)
normal development as male till puberty then taller height wider hips and gynaecomasitia weaker muscles small testes reduced libido shyness infertility subtle learning difficulties
627
what hormone differences are there in klinefelter's syndrome?
Raised gonadotrophin Low testosterone
628
what is the management of klinefelters syndrome?
testosterone injections advanced IVF techniques breast reduction
629
what are 4 things that at at increased risks in klinefelters?
breast cancer (more than other men) osteoporosis diabetes anxiety and depression
630
what is turners syndrome?
females with single X chromosome on chromosome 45 -> 45XO
631
what are 9 features of turner syndrome?
short stature webbed neck high arching palate downward sloping eyes with ptosis broad chest and with wide spaced nipples cubitus valgus - exaggerated elbow angle underdeveloped ovaries late/incomplete puberty infertile
632
what are 9 conditions associated with turners syndrome?
recurrent otitis media recurrent UTI Cardiac - 1 - bicuspid aortic valve, 2- coarctation of aorta, pulmonary stenosis hypothyroid hypertension obesity diabetes osteoporosis horse shoe kidney learning difficulties primary amenorrhoea
633
what are 3 managements for turners?
growth hormone therapy for height oestrogen and progesterone for female secondary sex characteristics fertility treatment
634
what is the genetic abnormality in downs?
Trisomy 21
635
what are 8 dysmorphic features of downs syndrome?
Brachycephaly (small head with flat occiput) flattened face and nose prominent epicanthic folds Brushfield spots in iris protruding tongue Small low set ears upward sloping palpebral fissures single palmar crease and large sandal gap hypotonia short neck short stature
636
what are 8 complications of downs?
recurrent otitis media and deafness (eustachion tube abnormalities) visual problems hypothyroidism cardiac defects - ASD, VSD, PDA, tetralogy atlantoaxial instability leukaemia - AML (most common) and ALL Alzheimers Hirschprungs disease and duodenal atresia
637
what cardiac defects are more likely in downs?
ASD VSD PDA Tetralogy of fallot
638
what nuchal thickness is indicative of downs?
>6mm
639
what is the combinded test for downs syndrome?
11-14 weeks USS for nuchal translucency bloods - High B-HCG, Low PAPPA
640
what is the tripple test?
between 14-20 weeks maternal bloods - High B-HCG, Low alpha-fetoprotein Low serum oestrodiol
641
what is the quadruple test for downs syndrome?
14-20 weeks High HCG Low AFP Low Total Oestrodiol High inhibin-A
642
what are 2 ways of antenatal testing for downs?
chorionic villus sampling amniocentesis
643
what are 4 routine follow ups needsd in downs?
regular thyroid checks - 2 yearly echo regular audiometry regular eye checks
644
what is the average life expectancy for downs?
60 years
645
what is the genetics underlying patau syndrome?
trisomy 13
646
what genetics cause Edwards syndrome?
trisomy 18
647
What are 7 key features of Edwards syndrome syndrome?
Rocker(rounded)-bottom feet Overlapping fingers low set ears Cardiac and renal malformations low birth weight prominent occiput small mouth and chin short sternum
648
what are 6 features of Pataus?
Polydactyl cleft lip and palate structural defects of brain scalp defects small eyes and eye defects cardiac and renal malformations May also have rockerbottom feet
649
what gene is affected in fragile X syndrome?
fragile X mental retardation 1 gene on X chromosome males are always affected but females vary due to trinucleotide repeat
650
what are 8 features of fragile X?
intellectual disability long narrow face large low set ears large testes after puberty hypermobile joints ADHD ASD Seizures
651
what gene is affected in Angelman syndrome?
UBE3A gene on chromosome 15
652
what are key features of Angelman syndrome?
Developmental delay - severe delay/absence in speech Coordination and balance problems Fascination with water happy demeanour inappropriate laughter hand flapping abnormal sleep patterns epilepsy ADHD Dysmorphic features - microcephaly, fair skin, hair and blue eyes, wide mouth and widely spaced teeth
653
what is the genetics of prader-willi?
loss of functional genes on proximal arm of chromosome 15 inhertied from father
654
what are 6 key features of prader-willi?
constant insatiable hunger hypotonia mild/moderate learning disability hypogonadism dysmorphic - narrow forehead, almond eyes, thin upper lip, downturned mouth, squint mental health problems Fair soft skin, prone to bruising
655
what is the management of prader willi?
growth hormone - for improving muscle development an body composition dieticians, education support, psych, physio, OT
656
what is the inheritance of most of noonan syndrome?
autosomal dominant
657
what are 8 features of noonan syndrome?
short stature broad forehead - triangular shaped face downward sloping eyes with ptosis hypertelorism (widespaced eyes) prominent nasolabial folds low set ears webbed neck widely spaced nipples
658
what are 6 conditions associated with noonan syndrome?
congenital heart disease - pulmonary stenosis, HOCM, ASD Undescended testes learning disability lymphoedema increased risk of leukaemia and neuroblastoma
659
what is the cause of william syndrome?
deletion on chromosome 7 - usually random rather than inherited
660
what are 8 features of william syndrome?
broad forehead starburst iris flattened nasal bridge long philtrum wide mouth and spaced teeth small chin sociable and trusting personality mild learning disability
661
what are 4 conditions associated with william syndrome?
supravalvular aortic tenosis hypercalcaemia ADHD hypertension
662
what is the management for williams sydrome?
echo and BP monitoring low calcium diet
663
what is gower's sign?
children with proximal weakness get onto their hands and knees then go into downward dog and push themselves up using their hands MUSCULAR DYSTROPHY
664
what is the name of the sign that is the way children with muscular dystrophy get up?
Gower's sign
665
what are 7 types of muscular dystrophy?
duchennes muscular dystrophy beckers muscular dystrophy faciosapulohumeral muscular dystrophy oculopharyngeal muscular dystrophy limb-girdle muscular dystrophy emery-dreifuss muscular dystrophy
666
what is the inheritance for duchennes MD?
X-linked recessive
667
what is the genetic cause of duchennes muscular dystrophy?
defective dystrophin gene on X-chromosome due to frame shift Dystrophin holds muscles together at cellular levels
668
what is the presentation of Duchenne MD?
Progressive proximal muscle weakness from 2-5 years Calf pseudohypertrophy Gower's sign waddling gait 30% have intellectual impairment
669
what are 2 investigations for Duchenne MD?
Creatinine kinase genetic testing
670
what is the prognosis for duchennes MD?
25-35 years
671
what is the management of duchennes MD?
oral steroids - slow progress of muscle weakness creatine supplements improve muscle strength
672
when do symptoms start to appear in Duchenne MD?
3-5 years
673
what are 3 complications of Duchenne MD?
Dilated cardiomyopathy Respiratory failure - due to weakness of diaphragm Arrhythmias
674
when do symptoms start to appear in beckers MD?
8-12 years less severe than Duchenne Require wheelchair in adulthood
675
when does myotonic dystrophy usually present?
adulthood
676
what are 4 key features of myotonic dystrophy?
progressive muscle weakness prolonged muscle contractions cataracts cardiac arrhythmia
677
what are 4 key features of facioscapulohumeral muscular dystrophy?
weakness around face progressing to shoulders and arms sleeping with eyes slightly open weak pursing lips unable to blow out cheeks without air leaking from mouth Onset in childhood
678
what are 3 key features of oculopharyngeal MD?
bilateral ptosis restricted eye movements swallowing problems usually presents in late adulthood
679
what is limb girdle MD?
presents in teenage years with progressive weakness around limb girdles - hips and shoulders
680
what is emery-dreifuss MD?
presents in childhood with contractures which restricts range of movement also progressive weakness and wasting of muscles starting with upper arms and lower legs
681
what bacteria causes TB?
mycobacterium tuberculosis
682
what stain is required in TB?
Zeihl-neelson stain acid-fast bacilli that turn red against blue background on Z-N
683
what are 8 symptoms of TB?
cough haemoptysis lethargy fever/night sweats wt loss lymphadenopathy erythema nodosum spinal pain
684
what are 2 investigations for TB?
mantoux test interferon gamma release assay
685
what is the mantoux test?
for TB inject tuberculin proteins into intradermal space and measure size of injection site at 72 hours - >5mm = positive
686
what is the appearance of disseminated miliary TB on CXR?
millet seeds uniformally distributed
687
what is the treatment for active TB?
RIPE Rifampicin - 6 months Isoniazid - 6 months Pyrazinamide - 2 months Ethanbutol - 2 months
688
what is the treatment of latent TB?
isoniazid and rifampicin 3 months or isoniazid for 6 months
689
what are 3 side effect of rifampicin?
red/orange piss and tears reduced CYP450 drug effectiveness - COCP hepatotoxic
690
what should be prescribed with isoniazid?
pyridoxine (vit B6)
691
what are 2 side effects of isoniazid?
peripheral neuropathy hepatotoxic
692
what are 2 side effects of pyrazinamide?
hyperuricaemia => gout and kidney stones hepatotoxic
693
what are 2 side effects of ethambutol?
colour blindness reduced visual acuity
694
what are 3 risks of undescended testes?
testicular torsion infertility testicular cancer
695
what are 5 risk factors for undescended testes?
FHx low birth weight small for gestational age prematurity maternal smoking
696
what is the management for unilateral undescended testes?
watch and wait for 3 months then refer orchidopexy between 6-12 months
697
what is the management of bilateral undescended testes?
urgent review within 24 hours
698
what are 6 presentations of congenital hypothyroidism?
usually picked up on blood spot prolonged neonatal jaundice poor feeding constipation increased sleeping reduced activity slow growth and development
699
what is a complication of congenital hypothyroidism?
irreversible cognitive impairment if not treated within first 4 weeks of life
700
what antibody is present in hashimotos?
antithyroid peroxidase antibodies and antithroglobulin antibodies
701
what causes congenital adrenal hyperplasia?
congenital deficiency in 21-hydroxylase enzyme causing underproduction of cortisol and aldosterone and overproduction of androgens (testosterone) Causes increased sodium and potassium excretion
702
what is the inheritance pattern for congenital adrenal hyperplasia?
autosomal recessive
703
what is the pathophysiology of congenital adrenal hyperplasia?
lack of 21-hydroxylase which converts progesterone into aldosterone and cortisol progesterone can be converted to testosterone without 21-hydroxylase enzyme therefore all progesterone is converted to testosterone instead of aldosterone and cortisol
704
what is the presentation of congenital adrenal hyperplasia in severe cases?
enlarged clitoris in females hyponatraemia hyperkalaemia hypoglycaemia poor feeding vomiting dehydration arrhythmias SKIN HYPERPIGMENTATION
705
what is the presentation of congenital adrenal hyperplasia in mild cases?
female - tall for age - facial hair - absent periods - deep voice - early puberty Males - tall or age - deep voice - large penis - small testicles - early puberty
706
what investigations can be done for congenital adrenal hyperplasia?
serum 17-hydroxyprogesterone levels serum electrolytes - hyponatraemia, hyperkalaemia, acidosis serum hormone levels genetic testing
707
what is the management of congenital adrenal hyperplasia?
cortisol replacement - hydrocortisone aldosterone replacement - fludrocortisone virilised genitals corrective surgery
708
what are 6 complications of congenital adrenal hyperplasia?
adrenal crisis infertibility short stature peripheral precocious puberty metabolic abnormalities psychological issues
709
what is the inheritance pattern for androgen insensitivity syndrome?
X-linked recessive due to mutation on androgen receptor gene on X-chromosome in XY males
710
what is the pathophysiology of androgen insensitivity syndrome?
cells are unresponsive to androgen hormones due to a lack of androgen receptors leading to excess androgens which are converted to oestrogen resulting in female secondary sexual characteristics and external female phenotypes from birth. Internally there are testes in the abdo/inguinal canal but no internal female organs due to the testes releasing anti-Mullerian hormone
711
what are 2 presentations of androgen insensitivity syndrome?
inguinal hernias primary amenorrhoea
712
what will hormone bloods be like in androgen insensitivity syndrome?
Raised LH normal/raised FSH Normal/raised testosterone for a man Raised oestrogen for a man
713
what is the management for androgen insensitivity syndrome?
bilateral orchidectomy - testicular tumours oestrogen therapy vaginal dilators and surgery
714
what is a wilms tumour?
nephroblastoma one of the most common childhood malignancies
715
what age does Wilms tumours usually present in?
<5 years
716
what are 4 congential syndromes that increase risk of wilms tumour?
WAGR syndrome - wilms tumour, aniridia, genitourinary anomalies, mental retardation Denys-drash syndrome - wilms, renal failure, ambiguous genitalia Beckwith-weidmann syndrome - congenital overgrowth Hemihypertrophy - overgrowth on one side of the body
717
what are 7 presentations of Wilms tumours?
abdo pain painless haematuria lethargy fever HTN Weight loss Abdo mass
718
What is the first line Ix for a Wilms tumour?
USS abdo
719
what is the management of Wilms tumour?
refer for r/v in <48h if suspected surgical excision +/- nephrectomy +/- adjuvant chemo/radio
720
what is the prognosis for Wilms tumour?
up to 90% cure in early stages
721
what is the most common leukaemia in children?
1st - ALL 2nd - AML
722
what is the most common leukaemia affecting people with Down's?
AML
723
what age is the peak incidence of ALL?
2-3 years
724
what age is the peak incidence of AML?
<2 years
725
what are 4 conditions that are risk factors for leukaemia?
Down syndrome kleinfelter syndrome Noonan syndrome faconi's anaemia
726
what is the prognosis for ALL?
80% cure rate
727
what are 7 complications of chemo?
failure of treatment stunted growth and development immunodeficiency and infection neurotoxicity infertility secondary malignancy cardiotoxicity
728
what causes Idiopathic thrombocytopenic purpura?
type 2 hypersensitivity reaction that casues antibodies to target and destroy platelets either spontaneously or triggered due to viral infection
729
what age group does idiopathic thrombocytopenic purpura present in?
<10 years
730
what is the management for severe idiopathic throbocytopenic purpura?
prednisolone IVIG blood transfusion if required platelet transfusion may work temporarily
731
what are 4 complications of ITP?
chronic ITP anaemia intracrania and subrachnoid haemorrhage GI bleeds
732
what are 5 causes of anaemia in infants?
physiological anaemia anaemia of prematurity blood loss haemolysis twin-twin transfusion syndrome
733
what are 3 causes of haemolysis in neonates?
Haemolytic disease of the newborn Hereditary spherocytosis G6PD deficiency
734
what is physiological anaemia of infancy?
normal dip in haemoglobin at 6-9 weeks due to high O2 at birth
735
what are 4 reasons for anaemia of prematurity?
less time in utero getting iron from mum RBC production cant keep up with growth reduced EPO blood tests
736
what is the treatment for helminth infection?
albendazole or mebendazole
737
what can interfere with iron absorption?
acid from stomach is required to comvert iron into ferrous (Fe2+) form so PPIs can interfere with absorption also coeliac or crohns
738
what test can be used to identify haemolytic disease of the newborn?
direct coombs test
739
what deficiency causes haemophilia A?
factor VIII
740
what deficiency causes haemophilia B?
factor IX
741
what is the inheritance pattern for haemophilia?
X-linked recessive
742
what is von willebrand factor?
a glycoprotein important in platelet adhesion and aggregation (formation of platelet plug)
743
what are the 3 types of von-willebrand factor disease?
1 - partial deficiency 2 - reduced function 3 - complete deficiency
744
what is the acute management of von willebrand disease?
desmopressin (stimulated release of vWF from endothelial cells) Tranexamic acid vWF infusion +/- factor VIII
745
what is faconi anaemia?
rare inherited disorder of gradual bone marrow failure and birth defects eventually leading to aplastic anaemia
746
what is kallmann syndrome and 3 features?
an X linked disorder Causes hypogonadotrophic hypogonadism due to failure of GnRH release Delayed puberty Low sex hormones, LH and FSH very low ANOSMIA Clef lip/palate and visual/hearing defects are sometimes seen
747
what are the three key areas affected by Autism?
deficits in social interaction communication and behaviour
748
what are 6 possible social diferences in autism?
lack of eye contact delay in smiling avoids physical contact unable to read non-verbal cues difficulty establishing friendships no desire to share attention (play with others)
749
what are 4 communication diferences in autism?
delay, absence or regression in language lack of appropriate non-erbal communication difficulty with imaginative or imitative behaviours repetitive use of words or phrases
750
what are 6 behavioural differences in autism?
greater interest in things than people sterotypical repetitive movements intensive deep interests repetitive behaviours and fixed routines anxiety and distress with deviation from routine extremely restricted food preferences
751
what are 6 features of ADHD?
very short attention span quick moving from one activity to another quick loss of interest in task or inability to persist with challenging task constantly moving or fidgeting impulsive behaviour disruptive or rule breaking
752
what are 3 ADHD meds?
methyphenidate - ritalin dexamfetamine atomoxetine
753
what is the first line antidepressant in children?
Fluoxetine 10-20mg
754
what are 8 signs of dehydration?
appear unwell altered consciousness sunken eyes tachycardia tachypnoea reduced skin turgor dry mucous membranes decreased urine outpt
755
what is the first line maintenance fluid choice in children >28 days?
0.9% NaCl + 5% glucose
756
what is the first line fluid choice in neonate <28 days?
if well 10% dextrose if unwell seek advice
757
what is the formula for maintenance fluids in >28 days of life?
100 mk/kg/day 1st 10kg 50ml/kg/day 2nd 10kg 20 ml/kg/day >20kg
758
what is the maintenance fluid requirements for <28 days?
day 1 - 50-60 ml/kg/day 2 - 70-80 ml/kg/day 3 - 80-100 ,l/kg/day 4 - 100-120 ml/kg/day 5-28 - 120-150 ml/kg/day
759
what is the calculation for percentage dehydration?
(well weight - current weight)/well weight X100
760
how do you calculate fluid deficit?
% dehydration X weight (kG) X 10
761
how do you calculate total fluid requirement?
maintenance fluid + fluid deficit
762
what fluids should be given for paeds resucitation?
0.9% NaCl 10 ml/Kg <10 mins
763
what is the normal ages to start to develop secondary sexual characteristics in males and females?
males - 9 years females - 8 years development before this age is precocious puberty
764
what are are 8 complications of obesity?
Slipped upper femoral epiphysis (SUFE) idiopathic intracranial hypertension hypoventilation syndrome fatty liver disease T2DM PCOS HTN abnormal blood lipids
765
what medication can be given to severely obese children >12 years?
orlistat
766
what are 4 causes of gonadotrophin (pituitary) dependant precocious puberty?
idiopathic/familial CNS abnormalities - congenital, tumours (neurfibromatosis, craniopharyngioma) Hypothyroism
767
what are 5 gonadotrophin independent causes of precocious puberty?
adrenal tumour congenital adrenal hyperplasia ovarian/testicular tumour (granulosa/leydig cell) Exogenous sex steroids
768
what age is usually affected by premature breast development (thelarche) absent of any other signs of precocious puberty?
6 months - 2 years fluctuating development of breast buds which is self limiting and does not require treatment
769
what is premature pubarche (adrenarche)?
when pubic hair develops precociously with no other signs of sexual development most commonly caused by accentuation of normal maturation androgen. More common in non-white population. Leads to increased risk of PCOS in adult life.
770
what is delayed puberty in males and females?
>14 years in females >15 years in males
771
what are 7 causes of delayed puberty?
congenital/familial - most common hypogonadotrophic hypogonadism Hypergonadotrophic hypogonadism
772
what are 4 causes of hypogonadotrophic hypogonadism?
systemic disease - CF, asthma, crohns Hypothalamo-pituitary disorders - tumours, kallman syndrome, pituitary dysfunction acquired hypothyroidism
773
what are 3 causes of hypergonadotrophic hypogonadism?
chromosomal abnormalities - klinefelter syndrome, turner syndrome steroid hormone enzyme deficiencies acquired gonadal damage
774
what is the most common solid tumour in children?
brain tumour
775
what is the most common brain tumour in children?
astrocytomas - raneg from benign to the highly malignant glioblastoma multiforme
776
what is the clinical presentation of brain tumour?
persistant/recurrent vomiting problems with balance, coordination or walking behaviour change abnormal eye movement seizures abnormal head positioning headache blurred/double vision lethargy deteriorating school work/developmental delay increasing head size in infants
777
what is medulloblastoma?
2nd most common childhood brain tumour arises in midline of posteriod fossa and may spread through CSF to spinal mets
778
what is an ependymoma?
brain tumour that behaves much like medulloblastoma but arises in posterior fossa
779
what is a brainstem glioma?
malignant brain tumour with very poor prognosis
780
what is a craniophryngioma?
developmental tumour arising from squamous remnant of rathke pouch - non-malignant but locally invasive
781
what age group is affected by neuroblastoma?
<6 years
782
where do neuroblastomas arise from?
neural crest tissue in adrenal medulla and sympathetic nervous system
783
what is the presentation of neuroblastoma?
mostly abdo mass but primary tumour may be anywhere along sympathetic chain from neck to pelvis pallor, wt loss, abdo mass, hepatomegally, bone pain, limp, proptosis
784
what investigations can be does for neuroblastoma?
urinary catecholamine metabolite levels (VMA/HVA) BIOPSY
785
what congenital condition is associated with increased risk of neuroblastoma?
noonan syndrome
786
what is the inheritance pattern for retinoblastomas and what chromosome is it found on?
autosomal dominant - incomplete penetrance chromosome 13
787
what are 2 presenting features of retinoblastoma?
red reflex turns white (leukocoria) squint ocular inflammation and redness vision loss
788
what is the management of retinoblastoma?
systemic chemo local therapies - laser photocoagulation or thermotherapy enucleation - removal of eyeball orbital exenteration radiotherapy
789
what is the most common liver tumour in children?
hepatoblastoma usually presents with bloating and abdo mass
790
what serum maker an be used for hepatoblastoma?
Alpha fetoprotein
791
when does the palmar reflex usually disappear?
2-6 months
792
when does the sucking reflex usually start?
around 32 weeks gestation - not fully developed until 36 weeks
793
how long does the moro reflex last?
around 2 months
794
how long does the stepping reflex last?
around 2 months
795
how long does the rooting reflex last?
around 4 months
796
what does use of NSAIDs in chicken pox increase risk of?
necrotising fasciitis - increase risk of bacterial infection
797
what is the infectivity period of chicken pox?
from 4 days pre-rash to around 5 days after rash appears when all crusted over
798
when is the meningitis B vaccine given?
2 months, 4 months, 12 months
799
what is the proper name for threadworms?
enterobius vermicularis
800
what is the management for threadworms?
Mebendazole single dose for whole household only for >6 months
801
what does a blood gas with pyloric stenosis look like?
Hypochloraemia Hypokalaemia elevated bicarb
802
what are 5 causes of obesity in children?
downs syndrome prader-willi syndrome growth hormone deficiency hypothyroid cushings syndrome
803
what are 5 consequences of obesity in children?
Ortho - SUFE, MSK Pains, Blounts disease Psycho - bullying, low self esteem sleep apnoea benign intracranial hypertension long term - T2DM, hypertension, IHD
804
what is the management for infantile spasms?
Prednisolone Vigabatrin
805
what inheritance pattern is there in achondropasia?
autosomal dominant
806
what mutation causes achondroplasia?
mutation in the fibroblast growth factor receptor 3 (FGFR-3) gene
807
what are 5 features of achondroplasia?
short limbs (rhizomelia) with shortened fingers (brachydactyly) large head with frontal bossing and narrow foramen magnum midface hypoplasia with a flattened nasal bridge 'trident' hands - gap between middle and ring finger lumbar lordosis
808
what is the management of asthma <5 years?
SABA + ICS for 8 weeks + leukotriene receptor antagonist (LTRA) refer to secondary care
809
what is a paeds ICS low dose?
<200 micrograms
810
what is a paeds ICS high dose?
>400 micrograms
811
what are 2 conditions associated with hypospadias?
cryptorchidism inguinal hernia
812
what is a venous hum?
innocent murmur heard just below clavicles of blood returning to heart
813
what is stills murmour?
innocent murmur of Low-pitched sound heard at the lower left sternal edge
814
what are 8 features of innocent murmurs?
soft-blowing murmur in the pulmonary area or short buzzing murmur in the aortic area may vary with posture localised with no radiation no diastolic component no thrill no added sounds (e.g. clicks) asymptomatic child no other abnormality
815
what is the management of asymptomatic neonatal hypoglycaemia?
encourage feeding + monitor
816
what is the management of symptomatic neonatal hypoglycaemia?
SCBU IV 10% dextrose
817
Is perthes usually unilateral or bilateral?
unilateral - only bilateral 10% of time
818
How is perthes disease diagnosed?
Bilateral hip X-rays
819
what vaccines are given at 8 weeks?
6 in 1 (DTaP, IPV, Hib, Hep B), Men B Rotavirus
820
what vaccines are given at 12 weeks?
6 in 1 (DTaP, IPV, Hib, Hep B) Pneumococcal Rotavirus
821
what vaccines are given at 16 weeks?
6in1(DTaP,IPV,Hib,HepB) MenB.
822
what vaccines are given at 1 year?
Hib/Men C Pneumococcal booster MMR Men B booster.
823
what vaccines are given at 3 years (4 months)?
DTap/IPV - Diptheria, Tetenus, Petussus, Polio MMR.
824
what vaccine is given at 12-13 years?
HPV
825
what vaccines are given at 14 years?
Tetanus, diphtheria, polio Men ACWY.
826
what is in the 6 in 1 vaccine?
Diphtheria Tetanus Pertussis Hib Hepatitis B Inactivated Polio Vaccine
827
when is the 6 in 1 vaccine given?
8, 12 and 16 weeks
828
when is the MMR vaccine given?
12 months and 3 years
829
When is the rotavirus vaccine given?
8 and 12 weeks
830
when is the men B vaccine given?
8 and 16 weeks booster at 1 year
831
what are the two main sanctuary sites for leukaemia?
CNS Testes most likely to have secondary malignancies
832
what is DiGeorge syndrome and it's features?
22q11.2 deletion congenital heart disease (e.g. tetralogy of Fallot), learning difficulties, hypocalcaemia, recurrent viral/fungal diseases, cleft palate
833
what is the name of deep rapid breathing in acidosis to rid body of CO2?
kassmaul breathing - seen in DKA
834
what is benign rolandic epilepsy?
twitching numbness or tingling episodes usually at night or when tired between ages of 4-12 years Can develop to tonic clonic child otherwise normal and has normal prognosis EEG has centrotemporal spikes
835
When does neonatal hypoglycaemia require treatment?
if symptomatic or BM <1 mmol/L
836
what is the mangement of neonatal hypoglycaemia?
2.5 mg/Kg 10% dextrose
837
what is the management of foecal impaction?
1 - macrogol laxative (movicol) 2 - senna if doesnt work after 1 week use lactulose if macrogol isn't tolerate
838
what surgery is done in Hirschprungs?
Swensen's procedure
839
what is the most common cause of convergent squint?
hypermetropia (long sightedness)
840
what extra feature is present in pentalogy of Fallot?
ASD
841
what is a discoid meniscus?
A developmental abnormality causing a disc shaped meniscus usually on the lateral side
842
what is the presentation of discoid meniscus?
locking or popping of knee along with pain and swelling
843
what is the management of discoid meniscus?
arthroscopic partial meniscectomy
844
what are 8 medications that can cause neonatal abstinence syndrome?
Opioids methadone benzos cocaine amphetamines nicotine or cannabis alcohol SSRIs/Antidepressants
845
when does opioid, diazepam, SSRI and alcohol withdrawal occur in neonates?
3-72 hours after birth
846
when does methadone and longer acting benzos withdrawal occur in neonates?
24 hours - 21 days
847
what are 6 CNS signs of neonatal abstinence syndrome?
Irritability increased tone high pitched cry not settling tremors seizures
848
what are 4 vasomotor and resp symptoms of abstinence syndrome?
yawning sweating unstable temp tachypnoea
849
what are 4 metabolic manifestations of abstinence syndrome?
poor feeding regurg and vomiting hypoglycaemia loose stools and sore nappy area
850
what are 2 oral treatments that can be given for abstinence syndrome?
opioid withdrawal - oral morphine sulphate non-opiate withdrawal - phenobarbitone
851
what most common bowel atresia?
jejunoileal atresia
852
what are 8 causes of intestinal obstruction in children?
meconium ileus hirschprungs disease oesophageal atresia duodenal atresia intusseption imperforate anus malrotation of intestines - volvulus strangulated hernia
853
what are 3 neutrophil disorders that can cause primary immunodeficiency?
chronic granulomatous disease chediak-higashi syndrome leukocyte adhesion deficiency
854
what are 3 beta cell disorders leading to primary immunodeficiency?
Common variable immunodeficiency Bruton's congenital agammaglbulinaemia selective immunoglobulin A deficiency
855
what T cell disorder can cause primary immunodeficiency?
DiGeorge syndrome
856
what are 4 combined B and T cell disorders that can cause primary immunodeficiency?
severe combined immunodeficiency ataxic telangiectasia wiskott-aldrich syndrome Hyper IgM syndromes