Paeds Flashcards

1
Q

name the 3 shunts in foetal circulation

A

foramen ovale
ductus arteriosus
ductus venosus

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2
Q

name the 4 left to right shunts

A

atrial septal defect
ventricular septal defect
atrio-ventricular septal defect
patent ductus arteriosus

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3
Q

describe an ASD and what are the most common types

A

hole connecting the 2 atria = leads to a shunt of blood moving from LA to RA = high pressure blood to lungs = RH failure and overload + pulmonary hypertension
ostium secundum = 80%
ostium primum = partial ASD

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4
Q

what are the symptoms of ASD

A

often asymptomatic in childhood or:
- sob
- difficulty feeding
- poor weight gain
older:
- recurrent chest - infections/wheeze
- heart failure

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5
Q

what are the clinical signs of ASD

A

fixed and widely split 2nd heart sound = pulmonary/aortic valves close at different times
ejection systolic murmur at left sternal edge in pulmonary area
possible palpitations

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6
Q

what investigations for ASD

A

chest XR = cardiomegaly wiht dilatation of pulmonary arteries
ECG = partial RBBB + right axis deviation
(partial ASD = left axis deviation)
echoCG = mainstay = shows anatomy

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7
Q

what is the management of ASD

A

small/asym = W&W
surgical closure/catheter closure
Anticoags in adults to prevent stroke complication

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8
Q

what is the most common cause of meningitis in the UK

A

neisseria meningitidis

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9
Q

what are the sepsis 6

A

WITHIN AN HOUR
1. high flow oxygen
2. blood cultures
3. obtain IV access + give IV ceftriaxone 80mg/kg
4. fluid resus of 20ml/kg 0.9% saline
5. measure lactate
6. measure urine

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10
Q

what is klinefelter syndrome and what are its features

A

XXY chromosomes = primary hypogonadism
- azoospermia + gynaecomastia
- reduced secondary sexual characteristics
- osteoporosis
- tall
- reduced IQ
** increased risk breast cancer

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11
Q

what is hypogonadotrophic hypogonadism

A

deficiency of LH and FSH

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12
Q

what is hypergonadotrophic hypogonadism

A

lack of response to LH/FSH by the gonads

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13
Q

what is the cause of hypOgonadotrophic hypogonadism and name some causes

A

= abnormal hypothalamus/pituitary function
- damage
- GH deficiency
- hypothyroidism
- hyperprolactinaemia
- serious chronic conditions (IBD/CF)
- excessive diet/exercise
- Kallman syndrome

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14
Q

what is the cause of hypERgonadotrophic hypogonadism

A

= gonads no response = no negative feedback = increased LH/FSH
- previous gonad damage
- congenital absence of gonads
- kleinfelters syndrome XXY
- turners syndrome XO

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15
Q

what is kallman’s syndrome

A

hypogonadotrophic hypogonadism
anosmia (no smell)
visual problems
craniofacial abnormalities

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16
Q

what is turners syndrome

A

45 X0
- often results in miscarriage
- lymphoedema hands/feet in neonate
- short stature (height 20cm below normal)
- gonadal failure
- webbing of neck/low hairline/big ears/high arched palate
- CVS malformations
- horseshoe kidney

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17
Q

name the causes of short stature

A

familial
constitutional delay in growth and development
malnutrition
chronic disease (IMD/CHD)
endocrine (hypothyroidism)
genetics (downs/turners)
skeletal dysplasia

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18
Q

how is constitutional delay in growth and puberty (CDGP) diagnosed

A

history/examination
XR of hand/wrist
bone age compared to actual age

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19
Q

name the causes of overgrowth with impaired final height

A

precocious puberty
congenital adrenal hyperplasia
hyperthyroidism
mcAlbright

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20
Q

name the causes of overgrowth with increased final height

A

androgen deficiency/oestrogen resistance
GH excess
Kleinfelter sydrome XXY/Marfan syndrome

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21
Q

what is psycho social short stature

A

seen over 3
emotional rejection/abuse associated
50% show reversible GH deficiency

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22
Q

what growth centile constitutes overweight and obese

A

overweight = BMI above 85th percentile
obese = BMI above 95th percentile

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23
Q

describe puberty in girls

A

breast bud first sign
early = under 8
late = over 13

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24
Q

describe puberty in boys

A

testicular enlargement first sign in boys
early = under 9
late = over 14

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25
what genetic syndromes can cause short stature
turners noonans downs russel-silver
26
causes of microcephaly
= familial = autosomal recessive condition = congenital infection = acquired after insult to brain e.g. meningitis
27
causes of macrocephaly
= familial = raised intercranial pressure = hydrocephalus = chronic subdural heamatoma = tumour = neurofibromatosis
28
causes of precocious puberty in girls vs boys
girls = usually early onset of normal puberty boys = organic cause - intracranial tumours - adrenal cause
29
what is congenital adrenal hyperplasia
autosomal recessive classic CAH = salt wasting type in boys 7-10 days = adrenal crisis ambiguous genitalia = cliteral hypertrophy and labial fusion
30
describe the important causes of vomiting in neonates (5)
malrotation/volvulus = bilious hirshprungs disease/meconium ileus = bilious NEC = bilious infection
31
describe the important causes of vomiting in infants (7)
GORD = feed associated food intolerance = change in stools pyloric stenosis = projectile intussusception = 3-36 months/colicky, red currant jelly stools strangulating hernia/obstruction = bilious raised ICP = early morning infection
32
describe the important causes of vomiting in older children (9)
appendicitis strangulated hernia pancreatitis DKA - diabetes symptoms meds/alcohol/drugs post-op pain psychiatric/eating disorder pregnancy infection - pyrexia
33
describe the investigations for acute vomiting
U&E stool virology abdo XR surgical consultation exclude systemic disease
34
describe the investigations for chronic vomiting
FBC ESR/CRP U&E LFT H.pylori serology urinalysis upper GI endoscopy abdo USS small bowel enema brain imaging test feed = pyloric stenosis
35
describe the investigations for cyclic vomiting
same as chronic +: amylase lipase glucose ammonia
36
what presentation is typical of pyloric stenosis
projectile vomiting at 2-7 weeks of age
37
what does bile stained vomiting suggest
intestinal obstruction intessuseption malrotation strangulated inguinal hernia
38
what are the complications of vomiting
K deficiency alkalosis Na depletion nutritional deficiency FTT anaemia malloey-weiss tear tear of small arteries dental erosions and caries oesophageal stricture/barrets oesoph
39
describe GORD in children
normal <12 months due to lower oesophageal sphincter immaturity increased risk in neuromuscular kids (cerebral palsy) effortless regurgitation usually self-resolving
40
what is the best diagnostic test for GORD
24hr oesophageal pH monitoring
41
describe the management for GORD
upright position feed thickeners medication = antacid/H2 blocker/PPI fundoplication if serious (rare)
42
what are the most common food allergens
cows milk eggs peanuts wheat soya fish shellfish tree nuts
43
what are the clinical features of food allergy/intolerance
diarrhoea +/- bloody/mucus vomiting abdo pain FTT eczema urticaria red rash, particularly around mouth asthma sympts/anaphylaxis if severe
44
what is the difference between a food allergy and an intolerance
food allergy = immunological response to food intolerance = adverse reaction mediated by NON-immunological response = more common
45
describe cow's milk protein allergy
most common allergy in infancy Sx depend on site of inflammation: upper GI = vomiting, pain small intestine = diarrhoea, pain, FTT large intestine = diarrhoea, acute colitis blood/mucus
46
what is the management of CMPA
limit cows milk protein intake *for mother if breastfeeding give formula if bottle fed dont give soya milk after 6-12months consider cows milk challenge
47
what is toddler diarrhoea
commonest cause of loose stool in preschool children undigested veg in stool common children are well and thriving no treatment
48
what is encoporesis
involuntary faecal soiling or incontinence = emotional disorder = more common in boys = can be secondary to constipation
49
what pattern indicates constipation
less than 3 stools per week hard, large stool
50
what is hirsprungs disease
absence of meyenteric plexus of rectum and colon usually presents in newborn as intestinal obstruction + delay in passing meconium w/n 24hr diagnose with suction rectal biopsy management is surgical
51
describe the red flags for constipation
no meconium w/n 48hrs = hirsprung/CF neurological signs vomiting = obstruction/hirscprungs ribbon stool = anal stenosis abnormal anus abnormal lower back/buttocks FTT acute severe abdo distention/pain/bloating
52
what is the most common cause of a UTI
E.coli
53
what would pseudomonas on a urine microscopy result suggest
structural abnormality
54
what is foaming urine indicative of
heavy protein loss in urine
55
name the acyanotic congenital heart diseases
left to right shunts: - atrial septal defect - ventricular septal defect - atrioventricular septal defect - patent ductus arteriosus Outflow obstruction: - coarctation of aorta - aortic stenosis - pulmonary stenosis
56
name the cyanotic congenital heart diseases
transposition of the great arteries tetralogy of fallot tricuspid atresia
57
describe the diagnostic criteria for anorexia nervosa
1. deliberately keeping weight below 85% expected = eating/exercise 2. dread of getting fat 3. endocrine effects = delayed menarche/menstruation stops
58
describe the epidemiology of anorexia nervosa
1 in 250 female 1 in 2000 males mean age of onset 16-17
59
describe the aetiology of anorexia nervosa
social pressure perfectionist character traits low self esteem reversing/halting effects of puberty family attitudes some genetic links depression/anxiety occupation (ballet) past or present events
60
describe the screening tool for eating problems
SCOFF do you make yourself SICK because uncomfortably full do you worry youve lost CONTROL over eating have you lost more than ONE stone in 3 months do you believe youre FAT when others say youre thin does FOOD dominate your life
61
describe the clinical signs of anorexia nervosa
dry skin lanugo hair orange skin/palms cold hands/feet bradycardia oedema postural hypotension weak proximal muscles
62
what is the mneumonic for appendicitis
MAGNET Migration of pain to RIF Anorexia Guarding Nausea Elevated temp Tenderness in RIF
63
what are the 2 most common causes of acute abdominal pain in children
non-specific pain appendicitis
64
how does malrotation typically present
within first few days of life (but can at any stage of life) obstruction green bilious vomiting blood in vomit = ischaemic bowel abdominal pain
65
what are the investigations and management for malrotation
urgent upper GI contrast study = barium meal treatment is urgent surgical correction (Ladd's procedure)
66
what is indicated with bilious or bloody vomiting in children
upper GI contrast study is needed
67
describe the presentation of intussuseption
2 months - 2 y/o paroxysmal/severe colicky pain pallor during pain episodes sausage shaped mass on examination redcurrant jelly stool - later presentation abdominal distension and shock
68
what is the most common cause of intestinal obstruction in infants after neonatal period
intussuseption - ileocaecal most common
69
what route for fluids if iv access cant be abtained
interosseous (into femur)
70
what antibiotic can you not give to neonates and why
ceftriaxone = causes jaundice
71
what causes purpura
skin microvessel thrombosis leading to heamorrhagic necrosis peripherals effected due to reduced perfusion to distal tissue in shock
72
what is the 1st line prophylaxis for meningitis
ciprofloxacin rifampicin also acceptable
73
describe the contraindications/complications for rifampicin
effect oral contraceptives cause jaundice person on anticoagulants person on seizure medication
74
what types of fluids can be used for maintenance in children
0.45% sodium chloride + 5% dextrose 0.9% sodium chloride + dextrose
75
what is the rate for maintenance fluids in children
100ml/kg for first 10kg 50ml/kg for next 10kg 20mls/kg for the rest
76
what fluid maintenance is given to babies on day 0
10% dextrose only
77
when is a fluid bolus given
hypoglycaemia - 10% dextrose 2ml/kg STAT hypovolaemia (shock) - 0.9% saline (even in neonate) 20mls/kg STAT
78
how is the deficit calculated for dehydration fluids
% dehydrated x 10 x weight in kg
79
advantages of breast feeding
macronutrients for baby free antibodies for infection free maternal bonding reduce NEC risk in preterm reduce risk of post menopausal breast cancers
80
disadvantages of breast feeding
unknown intake mother has to do all breast milk jaundice transmission of drugs/infections insufficient vit D/K in breastmilk
81
why is important to refeed slowly in anorexia
refeeding syndrome = body take up glucose and excrete sodium/potassium refeeding hepatitis
82
describe the diagnostic criteria for ADHD in under 17 year olds
6/9 inattentive symptoms 6/9 hyperactive/impulsive symptoms - present before 12 yr old - developmentally inappropriate - several symptoms in 2 or more settings - clear evidence symptoms intefere/reduce QoL (social/academic etc)
83
describe some features of inattentive symptoms
easily distracted not listening difficulty sustaining attention during activities forgetful in activities difficult to follow instructions difficult to organise tasks
84
describe some features of hyperactive symptoms
squirms/fidgets cant remain still run/climbs excessively 'on the go' talks excessively cannot do tasks quietly
85
describe some features of impulsive symptoms
blurts out answers to questions cannot wait their turn interrupts or intrudes on others
86
describe the epidemiology of ADHD
4-7% school age children males: females 4:1 overlaps with many other mental disorders
87
what are some risk factors for ADHD
foetal oxygen deprivation maternal smoking low birth weight prematurity heroin use in pregnancy genetic component low socioeconomic status other psychiatric disorders - aspergers/odd/tics
88
describe the non drug management of ADHD
parent education and training CBT to support social skills etc regular routines rewards and targets to reach for school support
89
describe the drug management of ADHD
stimulants (increase dopamine) = as needed: - ritalin = short acting - delmosart = long acting - elvanse non stimulants (breakdown norepinephrine) = regular taking: - atomoxetine (SNRI) - guanfacine ** or combination **
90
what is the efficacy of ADHD drugs
70% benefit of stimulant medication significant side effects - cardiac/anorexia/anxiety/depression
91
describe the prognosis of ADHD
30% grow out of it 15% remain meeting criteria by 25
92
describe the epidemiology of ASD
1 in 100 500,000 in UK boys x4 to girls
93
what are the cardinal symptoms of Autism SD
combination of difficulties of: 1. communication/speech and language 2. social interaction 3. behaviour rigidity/routines/rituals
94
describe some of the communication features of ASD
lack of desire to communicate communicate needs only disordered/repeated speech poor non verbal comms no social awareness
95
describe some of the social interaction features of ASD
no desire to interact lack of motivation to please others affectionate on own terms no understanding of social cues inappropriate touching/eye contact plays alone/stressful to be with others
96
describe some of the rigidity/imagination features of ASD
use toys as objects cannot play imaginatively resist change playing same game over/obsessions learn by rote not by understanding follows rules exactly inability to see others point of view
97
describe the management of ASD
education/information parenting workshops - timetables etc school liaison/support manage comorbidities
98
what are the features of an 'innocent' murmur
4 S's Soft blowing murmer aSymptomatic left Sternal edge Systolic murmur short duration
99
name the congenital infections to be aware of
CHRISTS CMV HSV Rubella hIV Syphillus Toxoplasmosis
100
what do you give a pregnant lady with syphillis
IM benzylpenecillin
101
how do you treat toxoplasmosis in pregnancy
spiromycin
102
what causes epiglottitis and what are some red flag signs
haemophilus B high fever drooling saliva soft stridor tripod stance/mouth open
103
how is epioglottitis managed
**do not examine throat** paeds emergency!!!!! anaesthetis and ENT surgeon needed keep child calm prepare for intubation once airway secures = blood cultures TX: cefuroxime 3-5 day abx
104
what is prophylaxis for epiglottitis
rifampicin for household contacts
105
what is the treatment for croup
1st line = oral dose dexamethasone severe: nebulised adrenaline oral prednisolone nebs steroids
106
what age group does bronchiolitis effect
up to 1 year old rare after 1
107
what preventative treatment is given for bronchiolitis
pavlizumab = for high risk - premy - CF - chronic heart/lung disease
108
what are the most common causes of pneumonia in newborn
group B strep gram negative enterococci = from mothers genital tract
109
what are the most common causes of pneumonia in infants/young kids
bacterial = strep. pneum = h.influenza = bordatella pertussis = chlamydia = s.aureus viral = RSV = rhinovirus
110
most common causes pneumonia in over 5s
strep. pneumonia chlamydia pneumoniae klebsiella mycoplasma
111
most common cause of pneumonia in immune comprpmised
pneumocystitis jiroveci TB
112
describe the indications for hospitalisation in pneumonia
sats <93% severe tachypnoea/difficult breathing grunting apnoea not feeding family unable to provide care
113
describe the antibiotic treatment for pneumonia
newborn = broad spec (benpen + gentamycin) older children = oral amoxicillin/erythromycin
114
describe the presentation of otitis media and what are the typical causative pathogens
irritable/miserable child pulling at ear 6 months - 12 months most common RSV rhinovirus pneumococcus h.influenzae
115
describe the examination findings of tympanic membrane for otitis media +/- effusion
red bulging lack of light reflection +effusion: = dull and retracted = visible fluid level
116
how is otitis media treated
simple analgesics 80% spontaneous resolution possible amoxicillin
117
what is glue ear and how is it treated
otitis media with effusion recurrent OM infections = reduce hearing most common cause conductive hearing loss in children no Tx or grommets
118
what are the most common causes of tonsillitis
viral group A beta haemolytic strep
119
what indicates more likely bacterial tonsilitis
headache apathy abdo pain tonsillar exudate/pus cervical lymphadenopathy
120
what are the causes of proteinuria in children
transient orthostatic increased glomerular perfusion pressure reduced renal mass hypertension tubular proteinuria glomerular abnormalities: GN, minimal change, abnormal BM
121
what is the most common cause of nephrotic syndrome in children + name 4 other causes
1. minimal change disease - HSP/vasculitis - SLE - infections - allergens
122
what are the 3 categories of nephrotic syndrome and who is high risk for each
congenital - finnish people steroid sensitive - boys/asian/atopic steroid resistant
123
describe congenital nephrotic syndrome
first 3 months of life rare but very serious end stage renal failure and dialysis needed high mortality
124
what are the features suggesting steroid sensitive nephrotic syndrome
age 1-10 no macroscopic haematuria normal BP normal complement normal renal function often resp infections precipitate
125
how is steroid sensitive nephrotic syndrome managed and what is the prognosis
60mg/m squared/day prednisolone 4 weeks every day 40mg/m squared/day 4 weeks alternate days relapses can occur if continue to adulthood may need methotrexate
126
what is the prognosis of steroid RESISTANT nephrotic syndrome
30% go to ESRF in 5 years 20% respond to cytotoxic meds most spontaneously remit in 5 years
127
describe the classification for FTT
mild = fall across 2 centile lines severe = fall across 3 centile lines
128
name the classification of causes of FTT
inadequate intake difficulty feeding malabsorption increased energy requirements inability to process nutrients
129
what are some organic causes of FTT
impaired suck/swallow - cleft palate/neuromotor dysfunction cardiac disease = breathlessness malabsorption = coeliac/CF/intolerance chronic illness = liver/heart/renal excessive calorie loss = vomiting/protein loss increased calorie need = malginancy/CF chromosomal = downs
130
what investigations for FTT
FBC U&E inflamm markers coeliac antibodies chest XR/sweat test for CF
131
what is the acute treatment for paediatric UTI
trimethoprim 3 day course or co-amoxiclav 5 day course fluids analgesia adjust abx once sensitivities return
132
what are the fraser guidelines
used to assess if underage person is competent to consent to treatment: 1. can understand advice 2. cannot be persuaded to inform parents 3. likely to continue sexual intercourse without protection 4. physical and mental health suffer if dont get contraception 5. its in her best interest to receive contraception wihtout parental consent
133
what is the definition of osteoporosis in children
1 + vertebral crush fractures 2+ long bone fractures by 10 3+ by 19 bone density <2.5 SD below mean
134
describe the causes of osteoporosis in children
inherited: - osteogenesis imperfecta - haematological acquired: - drugs (steroids) - endocrine disorder - malabsorption - inflammation/immobility
135
describe the pathophysiology of osteogenesis imperfecta
autosomal dominant type 1 collagen defect different types: type 2 fatal chest too small to allow breathing/multiple rib fractures/no lung function = brittle bones = fragiel bones
136
how does osteogenesis imperfecta present
bone fragility/fractures/deformity bone pain impaired mobility poor growth deafness/hernia/valvular prolapse
137
what is a distinctive feature of osteogenesis imperfecta
blue tinted sclera
138
describe the XR features of osteogenesis imperfecta
wormian bones (wiggly black lines in skull) = bubble wrap feeling bent bones bowing of femur complete chest collapse in type 2 fatal
139
what is the treatment for osteogenesis imperfecta
1. bisphosphonates = pamidronate - prefer given IV - increase lumbar spine/total body bone mass 2. vitamin D supplements 3. specialist MDT
140
describe the types of OI
type 1 mild type 2 fatal type 3 severe type 4 moderate
141
describe the role of vitamin D
- inreases Ca absorption from gut - increases Ca release from the bone - decreases Ca excretion by kidney - role in immune function
142
what is rickets and what are the risk factors
soft bones due to vitamin D or calcium deficiency can be genetic (X linked) insufficient vit D in pregnancy lack of sunlights dark skin lack of dietary vitamin D
143
how does rickets present
bowed leg hypocalcaemia convulsions gross motor delay swollen ankle carpopedal spasm incidental XR finding = splayed/frayed metaphyses
144
how is rickets/vitamin D deficiency treated
vit D supplement 400mg per day reccomended inactive form because they can convert it themselves
145
how is estimated weight calculated
under 12 months = (age in months +9)/2 1-5 =(age in years +4) x 2 5-14 = age in years x4
146
what is the wet flag mneumonic and what are the equations
Weight = (age+4)x2 Energy = 4J per kg Tube diameter = (age/4)+4 Fluid = 20ml x weight in medical, 10ml x weight in trauma Lorazepam = 0.1mg x weight Adrenaline = 0.1ml x weight of 1:10,000 Glucose = 2ml x weight of 10% dextrose
147
what are some causes of seizures in children
epilepsy febrile convulsions hypoglycaemia/hypocalcaemia head trauma infection (meningo/enceph/abcess) amphetamines/stimulants
148
describe febrile convulsions
6 months - 6 years high fever simple = <15 mins complicated = >15 mins may reoccur, no lasting damage
149
what are some causes of funny turns in children
breath holding attack reflexive anoxic seizures syncope migrain vertigo arrythmias fabricated by parent
150
describe the distinctive features of temporal lobe seizures
strange warning feelings/aura automatisms = lip smakcing/pacing deja vu and jamais vu impaired consciousness longer than absence
151
how are seizures investigated
witness history/video thorough history incl family history EEG blood tests metabolic investigations
152
what EEG features suggest absence seizures
3hrts spike and wave
153
what is the treatment for absence seizures
1. sodium valproate/ethosuximide 2. lamotrogine
154
what AED should be avoided in absence and myoclonic seizures
carbamezapine
155
what are the blood result criteria for DKA
BM over 11 bicarb under 15 ketones over 3
156
when are children's hearing checked
newborn starting school if parents are concerned
157
what are the 2 categories of hearing loss and the underlying pathology
conductive = obstruction in ear canal preventing sound from getting through, can be reversible sensorineural = nerve damage, irreversible
158
name some causes of conductive hearing loss in children (6)
congestion with cold most common glue ear ear wax middle ear infection perforated ear drum structural abnormality
159
name 3 risk factors of conductive hearing loss
down's syndrome craniofacial syndromes cleft palate
160
what is the exam finding of conductive hearing loss and how is it managed
better hearing through bone conduction self limiting ENT referral = grommets hearing aids if permanent cause
161
what are some causes of sensorineural hearing loss (7)
many unknown genetic/syndromal perinatal trauma/infection/hypoxia CMV/rubella in pregnancy meningitis premature babies
162
what is the examination findings in sensorineural hearing loss and how is it managed
hearing NOT better through bones on audiology higher frequencies worst heard = hearing aids = cochlear implants = aim to raise hearing so speech is audible
163
which cases are cochlear implants reserved for
profound hearing loss high frequency bilateral loss meningitis loss
164
what are some long term affects of hearing loss
developmental delay behavioural problems impact on education/friendships/social life
165
name 3 syndromes associated with hearing loss
ushers syndrome (sensorineural) wardenbergs syndrome (sensorineural) treacher-collins (conductive)
166
what congenital infection most commonly causes hearing loss
cytomegalovirus also: rubella syphillis herpes
167
when does hearing need to monitored
chemotherapy cystic fibrosis treatment CMV head trauma cleft lip/palate any of the syndromes associated with hearing loss
168
what is the difference between cyanotic breath holding spells and reflex anoxic seizures
cyanotic breath holding = stop breathing become blue and lose consciousness = after crying = involuntary = tired/lethargic afterwards reflex anoxic seizures = vagus nerve tells heart to stop beating = pale/unconscious/muscle twitches = regain conc within 30 secs = happens when child is startled/scared
169
up to what age is a squint normal
up to 12 weeks
170
what is strabismus and name some causes
misalignment of the visual axis refractive errors = most common cataracts retinoblastoma intra-occular problems
171
what are the 2 types of squint
non-paralytic/concomintant = refractive error paralytic = due to paralysis of motor nerves (rare)
172
what tests can be done to detect a squint
corneal light reflex test = reflection of light should be in same position in both eyes cover test = cover good eye and squinting eye will move
173
name the 3 refractive errors seen in childhood
hypermetropia (long sighted) myopia (short sighted) amblyopia (lazy eye)
174
what causes amblyopia and how is it treated
squint refractive errors ptosis cataracts treat early!! before 7 y/o = glasses = patching
175
how is strabisumus treated
glasses orthoptic exercises surgery = strengthen or weaken botox injections
176
what infection most commonly presents with neonatal jaundice + investigations
= UTI urine sample blood cultures CXR FBC
177
what type of jaundice in neonates is most worrying
persistent/prolonged
178
what features suggest biliary atresia
pale stools dark urine off feeds/unwell itching/scratch marks
179
what is the gold standard investigation for suspected biliary atresia
ultrasound liver
180
what counts as prolonged jaundice
>14 days in term babies >21 days in premature
181
what is an important complication of jaundice
kernicterus excess bilirubin causes brain damage non responsive/floppy/drowsy baby permanent damage = cerebral palsy/learning difficulties/deafness
182
what are the risk factors for neonatal hypoglycaemia
preterm IUGR diabetic mothers large babies polycythaemia unwell babies
183
how does neonatal hypoglycaemia present
jitters irritable apnoea lethargy/drowsiness seizures
184
how is neonatal hypoglycaemia managed
regular blood glucose bedside monitoring early and frequent milk feeding if need top up = IV dextrose via central venous catheter
185
describe an APGAR score
between 1 and 10 7-10 normal cardio resp colour reflexes tone
186
how does group B strep present in neonates and what is the mortality
pneumonia septicaemia meningitis (not as common) mortality = 10%
187
which women are high risk for GBS and are screened
pre-term delivery previous baby with GBS prolonged rupture of membranes fever over 38 in labour postpartum Abx given to high risk ladies
188
how is conjunctivits in the newborn managed
usually just water/saline if troublesome discharge could staph/strep = neomycin eye ointment gonococcal infection (48hrs after birth)= treat with penicillin or cephalosporin, cleanse frequently chlamydia (2 weeks after birth) = oral erythromycin for 2 weeks
189
what are the features of juvenile myoclonic seizures
occurs in the teen years early morning sudden myoclonic jerks = clumsy? triggered by lack of sleep often later develop generalised tonic-clonic (GTC) seizures may be inherited as autosomal dominant 40% with absence seizures will develop JME
190
a teenager spills their cereal every morning - what is this an indicator of
juvenile myoclonic epilepsy
191
what are the side effects of sodium valproate
teratogenic weight gain
192
what is ebstein's anomaly and when does it present
tricuspid valve is lower = larger RA, smaller RV = poor flow to pulmonary vessels associated with ASD = right to left shunt = cyanosis presents couple days post natal = when PDA closes
193
what are the clinical findings for ebsteins anomaly
ECG = arrhythmia = RBBB = left axis deviation CXR = cardiomegaly/RA enlarge echocardiogram = definitive diagnosis
194
how is ebsteins anomaly treated
treat arrythmia treat heart failure abx prophylaxis for IE surgical to correct
195
what findings are suggestive of a UTI
positive nitrites positive leucocytes just positive nitrites nitrites = better indicator of infection
196
describe the treatment of a UTI in an infant
all <3 months old with fever = IV abx and sepsis screen oral abx if over 3 months and well: - trimethoprim - nitrofurantoin - cefalexin - amoxicilin
197
describe the need for ultrasound for UTI
all under 6 months first UTI = abdo USS within 6 weeks children with recurrent UTI = abdo USS within 6 weeks children with atypical UTI = abdo USS during the illness
198
describe the risk factors for DDH
1st child breech presentation from 36 weeks breech at birth multiple pregnancy
199
name the tests to screen for DDH
ortolani test = abduct hips to see if hips dislocate anteriorly barlow test = pressure on knees/femur to see if femoral head dislocate posteriorly clicking = normal clunking = need ultrasound
200
how is DDH investigated
ultrasound of hips for all children with findings or risk factors XR useful in older children
201
describe the management for DDH
pavlik harness if under 6 months = keeps hips flexed and abducted regular review surgery if harness fails or >6 months old
202
describe the features of ulcerative colitis (CLOSE UP)
Continuous inflammation Limited to colon/rectum Only superficial mucosa Smoking is protective Excrete blood/mucus Use AMINOSALICYCLATES Primary sclerosing cholangitis
203
describe the features of Crohns (NESTS)
No blood/mucus Entire GI tract Skip lesions Terminal ileum most common and Transmural Smoking risk factor
204
what is pyloric stenosis and how does it present
hypertrophy and narrowing of pylorus = peristalsis of the stomach sends contents up oesophagus = vomiting in first few weeks of life - thin - pale - FTT - hungry - PROJECTILE vomiting firm round mass in upper abdomen (olive-like) metabolic alkalosis from loss of stomach acid
205
how is pyloric stenosis managed
Dx = abdominal USS laparoscopic pyloromyotomy (Ramsted's operation) = widens pylorus
206
what is gastroenteritis and what are the most common causes
inflammation from stomach to intestines with nausea, vomiting and diarrhoea most common causes = viral: rotavirus norovirus
207
what is biliary atresia and how is it treated
narrow or absent bile duct = cholestasis = no bile excreted = buildup of conjugated bilirubin = jaundice >14 days (term bb) >21 days (prem bb) treat with surgery = Kasai portoenterostomy = attach small intestine to liver often need full liver transplant
208
what is oesophageal atresia
congenital abnormality = blind ending oesophagus can have >1 fistulae connecting trachea as well = tracheo-oesophageal fistulae (TOF) associated with other anomalies = VACTERL syndrome
209
how does oesophageal atresia +/- TOF present
first few hours from birth: respiratory distress choking feeding difficulties frothing at mouth NG tube not possible overflow of saliva aspiration can occur
210
how is oesophageal atresia investigated and treated
CXR to assess lung fields + if theres TOF imaging renal tract = assess urogenital tract 'gap-o-gram' to assess length of oesophagus Mx: correct abnormality = surgery = supportive feeding and hydration
211
what is duodenal atresia and how does it present
gap or narrowing in duodenum present with bile green vomiting after feeds within first few days of life association with downs syndrome
212
how is duodenal atresia diagnosed and treated
abdo XR = 2 large air sacs (double bubble) surgical management
213
describe the causes of clubbing in children
hereditary infective endocarditis cyanotic heart disease cystic fibrosis liver cirrhosis IBD TB
214
what are the most common causative organisms for chest infections in cystic fibrosis
staph aureus = flucloxacillin long term to prevent pseudomonas = hard to treat, worse prognosis = tobramycin or ciprofloxacin
215
describe the management of hypoglycaemia in T1DM
too much insulin = low blood sugar pale/irritable/hungry/tremor/sweating treat with rapid acting insulin (lucozade) followed by long acting (toast/biscuits) severe = IM glucagon and IV dextrose nocturnal hypoglycaemia very common
216
describe the complication of hyperglycaemia in T1DM
BM too high but not DKA = alter insulin regimen
217
what is mesenteric adenitis
inflammation of lymph glands of the mesentery = umbilical/RIF abdominal pain common in children/teenagers fever/nausea/diarrhoea/cold sympts just before painkillers and supportive care
218
describe the pathophysiology of viral induced wheeze
children under 3 have smaller airways, smaller diameter virus = oedema and inflammation = airways constrict and narrow wheeze + lack of ventilation = respiratory distress
219
what is the difference between viral wheeze and asthma
viral wheeze: children <3 no atopic history only during viral infections
220
what is the presentation and management of viral induced wheeze
coryzal sympts roughly 2 days before wheeze shortness of breath signs of respiratory distress widespread expiratory wheeze management same as acute asthma management
221
what causes whooping cough and how does it present
bordatella pertussis gram neg bacteria coryzal sympts and dry cough then develop paroxysmal coughing attacks with loud inspiratory whoop coughing can cause fainting, vomiting, pneumothorax children may not make whooping sound
222
how is whooping cough diagnosed and treated
nasal/nasopharyngeal swab PCR tested/cultured cough >2 weeks = can test for anti-pertussis toxin IgG notify public health supportive care macrolides good in vulnerable people (erythromycin/clarithromycin etc) contact tracing
223
what is diptheria and how does it present
cornyebacterium diptheria causes mucosal membrane inflammation severe sore throat mild pyrexia lymphadenopathy tachypnoea grey membrane over tonsils
224
how is diptheria diagnosed and treated
throat swab = irregular gram +ve rods treat with antitoxin isolation for the individual to prevent spread
225
what is scarlet fever
disease caused by strep pyogenes (group A strep) highly contagious - aerosol or direct contact spread most common ages 2-8
226
how does scarlet fever present
sore throat/fever/nausea/vomiting pin point sandpaper-like blanching rash on trunk developing 12-48 hours after initial sympts - spreads to rest of body possible strawberry tongue + cervical lymphadenopathy
227
how is scarlet fever diagnosed and managed
clinical diagnosis oral antibiotics e.g. phenoxymethylpenicillin for 10 days notify public health isolation
228
describe the complications of scarlet fever
otitis media peritonsillar abcess acute sinusitis rheumatic fever acute post strep GN
229
what is congenital diaphragmatic hernia
birth defect of diaphragm = abdominal organs pushed into chest cavity = pulmonary hypoplasia and pulmonary HTN causes severe respiratory distress at birth Bochdalek most common type treated surgically to repair hernia while child is intubated 40-62% mortality rate
230
what is meconium aspiration syndrome
respiratory distress in newborn due to presence of meconium in the trachea if born in meconium stained amniotic fluid = at risk of developing diagnosis confirmed through CXR management is supportive ECMO and NO may be needed if persistent pulmonary HTN
231
what are some risk factors for meconium aspiration syndrome
gestation >42 weeks maternal Hx of HTN/preeclampsia/smoking/substance abuse foetal distress oligohydramnios
232
describe retinopathy of the newborn
retinal vessel growth stimulated by hypoxia if exposed to too much oxygen = excessive abnormal blood vessels and scar tissue form = causes retinal detachment
233
describe the diagnosis of retinopathy of the newborn
screening for all born <32weeks or <1.5kg performed by opthalmologist at 30-31 wk gestational age if born <27wks 4-5wk of age if born >27wk happen every 2 weeks until around 36 weeks gestation examination = retinal vessels and signs of plus disease
234
how is retinopathy of the newborn treated
transpupillary laser photocoagualation = stops new blood vessels developing cryotherapy also option
235
describe transient tachypnoea of the newborn
newborns present with tachpnea and respiratory distress due to delayes absorption of lung fluid following birth risk factors: - C sections - maternal asthma and smoking - premature infants - male sex - PROM frequently resolves within 72 hours
236
how is transient tachypnoea of the newborn diagnosed and treated
clinical diagnosis ABG to assess gas exchange pulse oximetry supportive treatment for resp distress rule out pneumonia and meconium aspiration
237
describe the causes of primary nocturnal enuresis
= child has never had a dry night overactive bladder fluid intake prior to bedtime failure to wake due to deep sleep/weak signals psychological distress and stress secondary
238
what is the management for primary enuresis
reassurance - probably resolve lifestyle changes - less fluid b4 bed etc encouragement and positive reinforcement treat underlying factors pharmacological tx
239
what is secondary enuresis and what are the causes
= child begins wetting bed after dry period of at least 6 months UTI constipation T1DM new psychosocial stress maltreatemnt **abuse/safeguarding**
240
describe the pharmacological treatments for enuresis
desmopressin = ADH = reduces volume of urine produced by kidneys oxybutinin = anticholinergic reducing contractility of bladder imipramine = tricyclin antidepressant may relax the bladder
241
what is nephritis and what is it caused by and what are the features
inflammation of nephrons caused by post strep GN and IgA nephropathy = nephritic syndrome 1. reduction in kidney function 2. haematuria 3. proteinuria (not as much as nephrotic syndrome)
242
describe the diagnosis and treatment of post strep glomerulonephritis
1-3 weeks post Beta strep infection e.g. tonisilitis supportive management with 80% full recovery impaired renal function = may need antihypertensives and diuretics for oedema
243
describe the diagnosis and treatement of IgA nephropathy causing nephritis
aka Berger's disease related to HSP renal biopsy will show IgA deposits usually teenagers/YA supportive management and immunosuppressent medications (steroids/cyclophosphomides)
244
what is status epilepticus and how is it managed
medical emergency = seizure >5 mins or >3 in and hour ABCD approach: - secure airway - oxygen - cardiac/resp functino - blood glucose - IV lorazepam - IV phenobarbital/phenytoin - transfer ITU
245
describe junior myoclonic epilepsy
occurs in teen years triggered by lack of sleep early morning myoclonic jerks (arms/shoulders) often later develop GTC seizures may be autosomal dominant inheritance
246
describe petit mal seizures
brief staring spells lasting a few seconds at a time abrupt onset and termination child may be unaware 1/3rd will have 1+ tonic clonic convulsions
247
describe acute chest syndrome and how it is managed
1. fever/resp sympts 2. new infiltrates seen on CXR can be infective or non infective cause = medical emergency with high mortality treat underlying cause: - antibiotics/antivirals - blood transfusions - incentive spirometry - artificial ventilation
248
whats the most common cause of meningitis in neonates
group B strep contracted during birth
249
how might meningitis present in a neonate/baby
non specific hypotonia poor feeding lethargy hypothermia bulging fontanelle
250
how is meningitis investigated
clinical examination kernig's test = lift bent leg then straighten from knee = pain brudzinski's test = gently lifting head and neck off bed = involuntary hip/knee flexion lumbar puncture
251
what level is a LP performed at
L3-L4 intervertebral space
252
describe the causes of acute glomerulonephritis
common: post infectious GN HSP IgA nephropathy less common: SLE Membranoproliferative GN focal segmental glomerulosclerosis
253
name the causes of headaches in children
tension migraines ENT infection analgesic headache vision problems raised intracranial pressure brain tumours meningitis/encephalitis carbon monoxide poisoning
254
describe tension headaches
very common in children band-like, symmetrical, gradual occur and resolve NO visual problems resolve within 30 mins triggered by stress/dehydration/hunger
255
how are migraines managed in children
rest/fluids/low stimulus environment paracetamol/ibuprofen sumatriptan antiemetics prophylaxis in severe cases: propranolol (CI in asthma) pizotifen (cause drowsy) topiramate (teratogenic)
256
what is abdominal migraine
common in young children - go on to develop migraines central abdo pain >1 hour normal examination nausea/vom anorexia headache pallor
257
what is gowers sign and when is it seen
to stand from lying down, child pushes hips up and out like downward dog and walks hands up legs = due to proximal muscle weakness in muscular dystrophy
258
what causes duchennes muscular dystrophy
defective gene for dystrophin on X chromosome = X linked recessive inheritance
259
describe DMD
boys around 3-5 with weakness around pelvis muscles progressive weakness life expectancy 25-35 cardiac and respiratory complications
260
describe beckers muscular dystrophy
dystrophin less severely affected presentation at 8-12 yr old longer life expectancy
261
describe myotonic dystrophy
genetic disorder presenting into adulthood progressive muscle weakness prolonged muscle contractions (unable to let go shaking hand) cataracts cardiac arrhythmias
262
what is facioscapulohumeral muscular dystrophy
progressive weakness around face, shoulders, arms sleep with eyes slightly open and unable to purse lips
263
what is emery dreifuss muscular dystrophy
presents in childhood **contractures** elbows/ankles progressive weakness/wasting of muscles starting in upper arms/lower legs
264
what is west syndrome
= infantile spasms rare disorder onset around 6 months old cluster of full body spasms ppor prognosis = 1/3rd die by 25 treat with prednisolone and vigabatrin
265
what is friedreich's ataxia
autosomal recessive neurodegenerative disease associated with cardiomyopathy/diabetes onset before adolescence - progressive ataxia - ascending weakness and loss - of vibration/proprioception - scoliosis - cardiomyopathy/arrhythmias
266
what presents identically to friedreichs ataxia
vitamin E deficiency
267
how is freidreichs ataxia diagnosed and managed
nerve conduction studies ECG/echocardiogram exclude vitamin E deficiency MDT treatment - mainly supportive with physios and occupational therapists treatment of cardiac issues life expectancy 40-50
268
what is neurofibromatosis and what is the difference between type 1 and 2
autosomal dominant genetic disorder causing tumours to form on nerve tissue type 1 (more common) = recklinghausens disease = neurofibromas grow on nerves and compress nerves/tissue type 2 = bilateral acoustic neuromas develop causing hearing loss
269
describe the difference in presentation between neurofibromatosis 1 and 2
type 1 cafe au lait spots freckling dermal fibromas nodular neurofibromas lisch nodules (eyes) short stature/microcephaly type 2 cafe au lait spots deafness vertigo tinnitus signs of raising ICP
270
how is neurofibromatosis managed
supportive and monitoring progress in all areas treat comlications e.g. HTN/epilepsy/ADHD
271
describe some risk factors for neural tube defects
family history chromosomal disorders inadequate folate intake folic acid antagonists (methotrexate) anti-epilepsy drugs
272
describe the types of neural tube defect
anencephaly = absence of major portion of brain, skull and cap = cranial end of neural tube fails to close cephaloceles = brain matter herniate through skull spina bifida occulta (most common) = neural tube fail to close at caudal end but isolated laminar effects = usually only tuft of hair etc spina bifida cystica = defect of lumbar/sacral vertebrae = can be incidental/myelomeningocele/asymmetrical LMN weakness and wasting and limb deformity
273
how are neural tube defects diagnosed
MRI/CT scan USS antenatally prenatal screening = measure maternal alpha fetoprotein second trimester USS most accurate
274
what is tuberous sclerosis and how does it present
wideranging multisystem disorder = harmatomas in many organs commonly brain/skin/kidney commonly present in childhood with SKIN changes and EPILEPSY renal angiomyolipomas/lung tumours cognitive and behavioural problems treat and support each problem individually
275
describe extradural haemorrhage in children
rare due to plasticity of skull = bleeding between dura and skull due to fractured temporal/parietal bone damaging middle meningeal artery history of trauma/head injury with loss of consc followed by lucid period before deterioration headahce/nausea/vomiting CT = lemon shape avoid LP may need mannitol to reduce intracranial pressure may need burr holes
276
describe subdural haemorrhage in children
bleeding between dura and arachnoid mater due to tearing of bridging veins between cortex often due to acceleration-deceleration of head/blunt head trauma = shaking baby/abuse headache/possible loss of conc CT (with contrast) = banana shape opthalmology for retinal haemorrhages if shaken baby suspected mannitol/burr holes/surgery craniotomy/clot evacuation
277
describe subarachnoid haemorrhage in children
rare! caused by trauma = ?child abuse severe headache sudden onset nausea/vomiting/loss of consc/neuro deficits CT = star shaped surgical intervention
278
what is hereditary spherocytosis and how does it present
RBC sphere shaped = autosomal dominant common in northern europe jaundice anaemia gallstones splenomegaly haemolytic crises = symptoms more severe aplastic crises = parvovirus = no bone marrow response so severe anaemia/jaundice
279
how is hereditary spherocytosis diagnosed and managed
family history and clinical features blood film = spherocytes MCHC = raised reticulocytes = raised due to rapid RBC turnover Tx: folate supplement and splenectomy removal of GB if stones persistant problem transfusions during severe crises
280
what is G6PD deficiency
= defect in G6PD enzyme more common mediterranean/middle east/african X linked recessive pattern = males affected causes crises triggered by: infections medications fava beans crises = RBC attacked by reactive oxygen species = haemolysis
281
how does G6PD deficiency present and diagnosed
** neonatal jaundice ** anaemia intermittent jaundice gallstones splenomegaly Heinz bodies on blood film = denatured Hb within RBC G6PD enzyme assay for diagnosis
282
how is G6PD managed
avoid triggers: antimalarial ciprofloxacin nitrofurantoin trimethoprim
283
what is thalassaemia
autosomal recessive genetic defect in protein chains of Hb alpha (chrom 16) = depletion of alpha chains = more severe if more genes affected = african/asian pop beta (chrom 11) = depletion beta chains = no. genes affects NOT = severeity = european pop
284
how can thalassaemia present
microcytic anaemia (low MCV) fatigue pallor jaundice gallstones splenomegaly (increased RBC breakdown) pronounced forehead/malar eminences (bone marrow expands to produce extra RBC)
285
how is thalassaemia diagnosed
FBC = microcytic anaemia haemoglobin electrophoresis = globin abnormalities DNA testing for genetic abnormality
286
how is alpha thalassaemia managed
monitor for FBC and complications blood transfusions splenectomy bone marrow transplant
287
how is beta thalassaemia managed
minor = monitor mild microcytic anaemia intermedia = significant anaemia = blood transfusions, may require iron chelation major = 2x deletion genes = severe anaemia and FTT = regualr transfusion/iron chelation/splenectomy/BM transplant
288
what is a common complication of thalassaemia
iron overload = monitor ferritin levels = limit transfusions and perform iron chelation effects: fatigue liver cirrhosis infertility impotence heart failure arthritis diabetes
289
what is haemophilia
inherited condition X linked recessive type A = more common = lack of factor VIII type B = lack of factor IX prothrombin time normal partial thromboplastin time increased presents with bleeding/bruising/haematomas e.g. prolonged umbilical bleeding in neonate mild to severe disease presentation
290
how is haemophilia managed
replace missing clotting factors with regular infusion type A = factor VIII type B = factor IX
291
what is idiopathic thrombocytopenic purpura ITP
spontaneous low platelet count causing purpuric rash caused by type 2 hypersensitivity reaction = antibodies destroy platelets can be triggered presentation: bleeding bruising petechial/purpuric rash (non blanching)
292
how is ITP managed
urgent FBC for plt count = confirm Dx exclude other causes e.g. leukemia usually no treatment required plt <10 or actively bleeding: prednisolone IV Ig blood transfusions platelet transfusion (only temporary)
293
what are the complications and advice for ITP
chronic ITO anaemia intracranial and subarachnoid haemorrhage GI bleeding = avoid contact sports = avoid NSAIDs/blood thinners/bleeding procedures
294
what is perthes disease
disruption of bloodflow to femoral head = avascular necrosis = affects epiphysis of femur 4-12 y/o with common 5-8y/o no clear cause revascularisation + healing of femoral head can lead to soft and deformed femoral head = early OA
295
how does perthe's disease present and how is it diagnosed
slow onset + no Hx of trauma pain in hip/groin limp restricted movement referred pain to knee - XR (can appear normal) - bloods to exclude other causes - technetium bone scan - MRI
296
how is perthes disease managed
severity varies conservative Mx in younger/milder bed rest traction crutches analgesia = reduce risk of damage/deformity when healing = physio/regular XR/surgery in severe cases
297
describe slipped upper femoral epiphysis and its presentation
head of femur displaced along the growth plate 8-15y/o (12y/o common) and boys>girls Px adolescent obese male undergoing growth spurt with Hx minor trauma - hip/groin/knee pain disproportionate to minor trauma - RoM hip - painful limp examination = prefer external rotation of hip with PAIN with internal rotation of hip
298
how is SUFE diagnosed and managed
bloods = normal/to exclude xr of both hips AP and frog leg = in situ fixation with cannulated screw = internal fixation across growth plate
299
describe osgood-schlatter disease
inflammation at tibial tuberosity at patella tendon insertion due to stress from activity and exercise = multiple small avulsion fractures = cause visible lump below knee = tender and inflamed pain in anterior knee pain worse by activity/pressure once healed = non tender and hard lump
300
how is osgood-schlatter disease managed
reduction in physical activity ice NSAIDs stretching/physiothreapy symptoms usually resolve - may have small lump under knee
301
what is chondromalacia patellae
= softening of articular cartilage of patella seen on MRI = patellofemoral pain syndrome inflammation and pain in and around patella caused by compressive forces from exercise or long periods of knee flexion common in sporty and active adolescents/YA treat with physio/analgesia
302
what is patellar tendonitis
= 'jumpers knee' inflammation at patellar tendon due to overuse adolescents = sinding-larsen johansson disease = chronic stress injury = anterior knee pain and tenderness XR may be normal USS examination of choice = shows swelling rest/analgesia/physio to treat
303
what is osteochondritis dissecans
pathological process affecting subchondral bone of children with open growth plates affects knee mainly trauma/male/overuse/family hx = risk factors = vague aching pain/oedema/formation of loose bodies in joint/latching and catching joint XR show loose body USS/CT showing lesions MRI best for evaluation Tx = analegesia/physio/surgery
304
what is achondroplasia
= dwarfism = FGFR3 gene on chromosome 4 = autosomal dominant inheritance homozygous gene mutation = fatal in neonatal period
305
what are the features of achondroplasia
disproportionate short stature (4 ft) short limbs short digits bow legs disproportionate skull foramen magnum stenosis (cervical cord compression/hydrocephalus) recurrent otitis media due to cranial abnormalities obesity obstructive sleep apnoea
306
what is molluscum contagiosum
viral skin infection caused by type of poxvirus small flesh coloured papules with central dimple appear in crops of lesions spread through direct contact/shared linens avoid scratching/breaking skin as can cause additional infection clear up spontaneously up to 18months can treat with potassium hydroxide/benzoyl peroxide/imiquimod surgical removal is option but can cause scarring
307
describe scabies
tiny mites (sarcoptes scabiei) burrow under skin cauing intense itching and small red dots rash finger webs common area easily spread so ask about contacts with itching treat with permethrin cream = need to be applied to entire body and left for 8-12 hours then washed off = repeat after a week oral ivermectin single dose option for difficult to treat all household needs treatment and deep clean all linens and carpets crotamiton cream for itching
308
what are crusted scabies
norwegian scabies serious infestation with scabies in immunocompromised can present like psoriatic plaques with no ithcing may require hospital treatment and isolation
309
what is impetigo
superficial bacterial skin infection caused by staph aureus also can be caused by strep pyogenes VERY infectious = no school during infection
310
what is non-bullous impetigo
occur around nose/mouth exudate dries = golden crust treat with topical fusidic acid oral flucloxacillin to treat more widespread advise measures to avoid spread
311
what is bullous impetigo
always caused by staph aureus = epidermolytic toxins that break down skin proteins = fluid vesicles form then burst = golden crust painful and itchy more common neonates + <2y/o can have systemic symptoms swabs to confirm Dx flucloxacillin treatment very contagious = isolation needed
312
what are the complications of impetigo
cellulitis sepsis scarring post strep GN staph scalded skin syndrome scarlet fever
313
what is erythema multiforme
erythematous rash caused by hypersensitivity reaction caused by viral infections and medications e.g. HSV/mycoplasma pneumonia widespread itchy red rash with target lesions sypmts abrupt onset + mild fever/stomatitis/muscle aches/flu-like sympts Tx underlying cause mild and should resolve spontaneously
314
what is staphylococcus scalded skin syndrome (SSSS)
staph aureus release toxins break down skin proteins = infection affects children <5 generalised patches of erythema --> fluid blisters (bullae) --> burst and scar = scalded Nikolsky skign = gentle rubbing of skin causes it to peel away = confirm SSSS + systemic symptoms tx by hospital admission + IV fluids and IV Abx
315
what is ringworm and what are the different types
fungal infection aka tinea or dermatophytosis tinea capitis = on scalp tinea pedis = foot/athletes foot tinea corporis = body tinea cruris = groin onchomycosis = fungal nail
316
how does ringworm present
itchy/erythmatous/scaly/well demarcated rash capitis = hair loss/dryness pedis = between toes ** check toes if ringworm = athletes foot common**
317
how is ringworm diagnosed and managed
clinical diagnosis + response to antifungals possible to scrape and culture treat with antifungals - clotrimaxole - miconazole - ketoconazole mild topical steroid help with inflammation and itching advice to avoid/prevent future infections
318
what is tinea incognito
more extensive fungal skin infections caused by use of steroids to treat fungal infection = suppresses immune response = infection grows
319
what causes chicken pox and how does it present
varicella zoster virus = highly contagious presents with fever first itch general fatigue/malaise rash - widespread/red/raised/vesicular once scabbed = not contagious
320
how is chicken pox spread
highly contagious spread through direct contact with lesions or infected droplets from cough/sneeze symptomatic 10 days03 weeks after exposure
321
what are the complications of chicken pox
bacterial superinfection dehydration conjunctival lesions pneumonia encephalitis virus can lay dormant in sensory dorsal root ganglion cells = later = shingles or ramsay hunt syndrome
322
how is chicken pox managed
mild/self-limiting aciclovir considered in immunocompromised patients/>14 presenting <24hr/neonate itching = calamine lotion/antihystamine
323
what causes hand foot and mouth disease and how does it present
coxsackie A virus 3-5 day incubation upper resp tract symptoms and fever 1-2 days = small mouth ulcers with blistering red spots across body (hand/foot/mouth) painful mouth ulcers + on tongue
324
how is hand foot and mouth disease managed and what are the complications
diagnosis = clinical appearance supportive management resolve in week-10 days highly contagious = careful dehydration/bacterial superinfection/encephalitis
325
what is roseola infantum
sixth disease/3 day rash caused by herpesvirus 6/7 high fever sudden development rash over body excluding face (often after fever subsides) sore throat lymphadenopathy supportive management
326
what is erythema infectiosum
aka slapped cheek syndrome caused by parvovirus B19 can be passed from mother to baby via placenta may be asymptomatic or non specific coryzal sympts symptom free 7-10 days then rash appear on cheeks can present with arthropathy management = symptomatic
327
what are the complications of erythema infectiosum
transient aplastic crisis in those with sickle cell/thalassaemia/hereditary spherocytosis/iron deficiency anaemia can cause immunocytopenia in immunodeficient patients can cause meningoencephalitis in immunocompromised DANGEROUS IN PREGNANCY
328
what are the features of osteosarcoma
= bone cancer 10-20 y/o most commonly femur (tibia/humerus) ** persistent bone pain, especially at night ** bone swelling/palpable mass/RoM
329
describe the diagnosis of osteosarcoma
very urgent direct access XR in 48 hours if unexplained bone pain/swelling if XR suggest osteosarcome - urgent specialist assessment 48hrs blood test = raised ALP CT MRI bone scan PET scan bone biopsy
330
describe the features of osteosarcoma on XR
poorly defined lesions in bone destruction of normal bone fluffy appearance periosteal reaction = sunburst appearance
331
how is osteosarcoma managed
surgical resection of lesion with limb amputation adjuvant chemo MDT team main complication = pathological bone fractures and metastasis
332
what is a wilm's tumour and how does it present
= nephroblastoma = cancer of kidneys presents 0-5 y/o asymptomatic abdo mass adbo pain haematuria UTI HTN
333
how is wilm's tumour diagnosed and managed
FBC renal function electrolytes urinalysis USS show distortion of renal pelvis renal angiography CT/MRI for staging Tx = nephrectomy followed by chemo routine postop radiotherapy is stage 3 prognosis = 90% survive long term increased risk of second tumours in wilms survivors
334
what is a rhabdomyosarcoma
malignancy of connective tissue where abnormal cells arise from primitive muscle cells most common around head/neck bladder (20%) muscles/limbs/chest (15-20%) = highly malignant and grow rapidly 87% patients are <15 (rarely affect adults)
335
what are the risk factors of rhabdomyosarcoma
neurofribromatosis fetal alcohol syndrome parental use of marijuana/cocaine exposure to XR
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how does rhabdomyosarcoma present
expanding lump/swelling = may be painful nose - nasal obstruction/discharge eyes = swelling eyeball adbdomen = pain/change in bowel habit bladder = haematuria
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how is rhabdomyosarcoma diagnosed
non resolving lumps in children = investigated for cancer bloods = anaemia/raised ALT urinalysis = haematuria XR/CT/MRI bone scan look for mets
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how is rhabdomyosarcoma managed
surgery for all lesions if possible chemo (vincristine/cyclophosphamide etc) post-op radiotherapy prognosis = 60% children/teens cured
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describe retinoblastoma
embryonal tumour of retina = most common eye malignancy in children inutero-5 y/o onset 40% = hereditary (AD)
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how does retinoblastoma present and what are the key differentials
abnormal pupil (leukocoria) strabismus deterioration of vision red/irritated eye Ddx: cataracts hamartomas choroiditis
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how is retinoblastoma diagnosed and managed
any child showing indication = red reflex performed exam under anaesthesia bidimensional ocular USS/MRI evaluate presence of mets genetic counselling Mx: urgent opthamology referral 1. surgery 2. chemo 3. radiation 99% survival in UK invasion of optic nerve most significant prognostic factor
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what are the most common types of brain cancer in children
astrocytoma most common = most are low grade, slow spread medullablastoma = most common high grade, fast spreading