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1
Q

management for minimal change disease

A

60mg/m2/day prednisolone
fluid restrict and low salt diet

non responsive after 4-6 weeks
- tacrolimus, ciclosporine

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2
Q

management of henloch schonlein purpura

A

Monitor:
Urineanalysis
BP
FBC, U&Es, creatinine

Most will resolve spontaneously after 4 weeks
joint pain managed with analgaesia eg. paracetamol, ibruprofen

sever abdo pain, scrotal involvement, severe oedema: prednisolone

nephrotic rane proteinurea: IV corticosteroids

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3
Q

Investigations and managament of suspected UTI

A

temp >38 and symptoms in all ages –> refer to paeds

symptoms in <3month –> urgent referral

urinalysis
+NIT +LEUK –> treat as UTI, send MSU for culture if <3y, not first uti, suspected pyelonephritis
+NIT -LEUK –> treat as UTI, send MSU for culture
-NIT +LEUK –> send MSU for culture, <3yrs start abx, >3yrs consider Abx if clinically indicated
-NIT -LEUK –> UTI unlikely

ABx:
simple UTI –> Trimethoprim or nitrofurantoin
upper UTI/ pyelonephritis –> Cefalexin, co-amoxiclav

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4
Q

Mx and counseling of coeliac

A

what is it:
condition where the body sparks an immune response to gluten causing damage to the gut
if gluten is eaten it can be very serious and will cause lots of pain, D+V and damage but more importantly increases risk of certain cancer

what now:
Gastro referral
investigations to confirm diagnosis
FBC- anaemia and deficiencies- replace
anti-TTG Abs, anti-enomesyial
duodenal biopsy- villous atrophy, crypt hyperplasia, lymphocyte infiltration

what this means in the future:
life long avoidance of gluten: wheat, barley and rye containing products
dietician referral
pneumococcal vaccination as hyposplenic
screen for other autoimmune conditions
annual reviews: height and weight, review Sx and adherence to diet

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5
Q

management of croup

A

single dose of dexamethosone
0.15mg/kg
A-E assessment: O2 if required

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6
Q

Webbed neck
Pectus excavatum
Short stature
Pulmonary stenosis
male or female

A

Noonans syndrome

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7
Q

Learning difficulties
Macrocephaly
Long face
Large ears
Macro-orchidism

A

fragile X

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8
Q

Micrognathia
Low-set ears
Rocker bottom feet
Overlapping of fingers

A

Edwards syndrome

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9
Q

Microcephalic, small eyes
Cleft lip/palate
Polydactyly
Scalp lesions

A

pataus syndrome

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10
Q

Micrognathia
Posterior displacement of the tongue (may result in upper airway obstruction)
Cleft palate

A

Pierre robinsons syndrome

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11
Q

Hypotonia
Hypogonadism
Obesity

A

Prader willi syndrome

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12
Q

Short stature
Learning difficulties
Friendly, extrovert personality
Transient neonatal hypercalcaemia
Supravalvular aortic stenosis

A

Williams syndrome

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13
Q

features of: Prader Willi syndrome

A

obesity
hypogonadism
hypotonia

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14
Q

features of: Noonan’s syndrome

A

pulmonary stenosis
pectus excavatum
wide spaced nipples
short stature

similar to turners but can be boys AND girls where as turners is just girls

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15
Q

features of: Edwards syndrome

A

Roker bottom feet
microganthia
lowset ears
overlapping of fingers

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16
Q

features of: Pataus syndrome

A

polydactyle
abnormal facies
clef lip and palate
small eyes
short gap between eyes

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17
Q

features of: Turners syndrome

A

short stature
shield chest, widely spaced nipples
webbed neck
bicuspid aortic valve (15%), coarctation of the aorta (5-10%)
primary amenorrhoea
cystic hygroma (often diagnosed prenatally)
high-arched palate
short fourth metacarpal
multiple pigmented naevi
lymphoedema in neonates (especially feet)
gonadotrophin levels will be elevated
hypothyroidism is much more common in Turner’s
horseshoe kidney: the most common renal abnormality in Turner’s syndrome

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18
Q

features of: Williams syndrome

A

short stature
LD
extroverted and friendly personality
transient neonatal hypercalcemia
supravalvular aortic stenosis

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19
Q

features of: Pierre Robinson

A
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20
Q

Features of: fragile X syndrome

A

LD
long face

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21
Q

definition of status epilepticus

A

prolonged seizure (>30 minutes) or recurrent seizures without return to baseline between seizures

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22
Q

causes of status epilepticus

A

Common:
febrile convulsions
epilepsy

rarer:
hyponatraemia
hypocalcaemia
CNS infection
hypoglycaemia
raised ICP
CVA
toxin poisoning
inborn error of metabolism

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23
Q

urgent investigations for status epilepticus

A

Investigations:
 Finger prick blood glucose
 FBC, sodium, calcium, magnesium, urea, creatinine, CRP
 Ammonia if neonate/ suspect inborn error of metabolism
 Consider toxicology screen (esp. teenagers)
 Blood pressure (exclude malignant hypertension)
 CT if focal signs/ new focal seizure, trauma, possible VP
shunt complication or space occupying lesion

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24
Q

Managament of status epilepticus

A

ABCD
A- secure airway, call anaethesist if not able to protect for intubation
B- give high flow oxygen HYPOXIA BIGGEST CONCERN
C- Insert cannulae, monitor BP
D- fingerprick glucose

  1. IV access –>IV lorazepam 0.1mg/kg (max 4mg)
    no IV access –> buccal midazolam 0.5mg/kg (max 10mg) OR rectal diazepam 0.5mg/kg
  2. continued to seize
    IV lorazepam 0.1mg/kg
    or paraldehyde PR 0.8mL/kg 50:50 mix (then consider IV/ IO access)
  3. CALL SENIOR HELP
  4. patient not normally on phenytoin –> give Phenytoin 20mg/kg by IV/IO over 20mins
    usually on phenytoin –> Levetiracetam 30mg/kg IV/IO(max 3g)
  5. CALL ON CALL ANAETHESIST AND PICU
  6. Rapid sequence induction of anaesthesia: intubate and ventilate
    Propofol 2-4mg/kg IV

Important issues
 Glucose: aim for 4-8 mmol/L
 Hyponatraemia (Na <135)
if Na <135 mmol/L and still
seizing OR Na <130 mmol/L give
bolus 3 mL/kg 2.7% sodium
chloride
 Aim for temp <37oC
 Meningitis
Ceftriaxone 80 mg/kg IV
 Encephalitis: add aciclovir +
macrolide
 Raised ICP on CT or clinical
signs – Neuroprotect guide

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25
signs of mild hypoxic ischaemic encephalopathy
irritability startle reponses poor feeding hyperventilation
26
signs of moderate HIE
altered tone reduced movement seizures
27
causes of seizure in newborn
encephilitis/ meingitis moderate-severe HIE hypoglycaemia hyponatraemia (eg. CAH) other electrolyte imbalances breath holding attacks reflex anoxic seizures
28
diagnostic finding on EEG for absence seizure
3 spike waves per second in all leads
29
describe benign rolandic epilepsy
partial seizures -> twitching, numbness or tingling of the child's face or tongue, and may interfere with speech and cause drooling -> child usually aware during seizure seizures may become generalised usually occur early in the morning young children (6-8yrs) and tend to grow out of it by teens without treatment may affect sleep and performance in school
30
loss of consciousness differentials
reflex anoxic seizure (baby/ toddler after being frightened or eg. bump on head) BRUE (baby) chocking on feeds breath holding attacks (toddler- commonly after temper tantrum) epilepsy narcolepsy vasovagal syncope heart conditions eg. WPW, HOCM hypoglycaemia
31
inflamed demyelinating HYPERINTENSE lesions in the white matter with changes in vision and loss of power indicative and urinary incontinence indicative of..
Multiple sclerosis
32
differentials for space occuplying lesions with surrounding oedema
brain tumour eg. glioma, neuroblastoma TB brain abscess
33
findings associated with tuberous sclerosis
Ash leaf macule CT/MRI sub epyndemal calcifications and hypointense white matter lesions
34
adverse risks in sickle cell disease
sickle cell painful crisis- blocked vessels leading to painful ischaemia acute chest syndrome (usually brought on by infection) avascular necrosis blood clots- MI, stroke, DVT, PE dactylitis - painful swollen fingers infection- increased risk of infection due to damage of the spleen eg. flu, meningitis, pneumonia (must have pneumococcal, flu and meningococcal vaccines) splenic sequestration- splenomegaly and pain from pooling of trapped blood
35
what is sickle cell disease
autosomal-recessive single gene defect in the beta chain of haemoglobin, which results in production of sickle cell haemoglobin (HbS).
36
management of acute painful (vaso occlusive) crisis in SCD
1. analgaesia eg. paracetamol/ibruprofen/ naproxen (caution with liver or kidney damage) Coedine not recommended in young children CI in <12y 2. O2 if required 3. correction of any blood gas disturbance 4. fluids if intravascularly depleted 5. blood exchange for life threatening
37
management of acute chest crisis in SCD
1. secure airway 2. give oxygen if required 3. broad spectrum antibiotics if infection after sending blood cultures 4. paracetamol for pain 5. fluids if necessary
38
side effect of sodium valporate
increased appetite weight gain hair loss liver failure
39
side effects of carbamazepine
PATCH Potentiates ADH -> water retention Aplastic anaemia/ agranulocytosis Teratogenic, transient leukopenia CNS effects- eg. dizziness, ataxia, headache, visual disturbances Hepatic transaminases elevated
40
side effects of lamotrigine
Rash behavioural changes irritability
41
side effects of vigabatrin
behavioural changes retinopathy sleep disturbances weight gain
42
side effeects of levetiracetam
anorexia abdominal pain vomiting diarrhoea behavioural changes thrombocytopenia
43
features of sturge weber syndrome
portwine stain on face neurological defects - leptomeningeal angioma, seizures glaucoma
44
skin lesions and associated condition: ash leaf macule cafe au lait spots portwine stain
ash leaf macule - tuberous sclerosis cafe au lait spots - neurofibromatosis, McCune albright portwine stain - Sturge-Weber syndrome
45
nocturnal eneuresis management
1. <5 reassure that will probably grow out of it look for possible causes: - DM - constipation - UTI - high fluid intake 2. positive reinforcement eg. gold stars, go to toilet before bed 3. eneuresis alarm 4. referral to specialist 5. desmopressin
46
what would be expected in each domain in a 6 week old
gross motor - lifts head 45 degrees from prone fine motor - holds rattle if given visual - fix and follows 180C speech and language - startles at loud noise social- smiles
47
what would be expected in each domain in a 3 month old
gross motor -Lying on abdomen, good head control fine motor -Reaches for object Holds rattle briefly if given to hand Visually alert, particularly human faces Fixes and follows to 180 degrees speech and language -Quietens to parents voice Turns towards sound Squeals social- Laughs Enjoys friendly handling
48
what would be expected in each domain in a 6 month old
gross motor - sits unsupported back curved, rolls from prone to supine fine motor - passess object from hand to hand visual - points at object speech and language - understands bye bye, no babbles turns head at loud sounds social- put things in mouth (stops at 1yr), shaked rattle
49
what would be expected in each domain in a 9 month old
gross motor - sits with straight back, walks with support fine motor - immature pincer grip visual - object permanence speech and language - social- scared of strangers (6months- 2 yrs) bites food
50
what would be expected in each domain in a 12 month old
gross motor - walks unsupported fine motor - mature pincer grip, tower of 2 bricks visual speech and language - understands nouns ('wheres mummy') 3 words points to body part social- waves bye bye, plays alone, drinks from beaker with lid
51
what would be expected in each domain in a 18 month old
gross motor - runs and jumps. red flag for not walking fine motor - tower of 4 visual speech and language - 1-6 words social- imitates everyday activities
52
what would be expected in each domain in a 2 year old
gross motor - up and down stairs two feet per step walk on tip toes throws ball fine motor- tower of 8, turns multiple pages of book at a time, draws vertical line (horizontal at 2.5yrs) visual speech and language - 2 words together, 50 word repertoire social- plays but doesnt share
53
what would be expected in each domain in a 3 year old
gross motor - hopping, walks upstairs one foot per step, downstairs two feet per step fine motor - builds bridge with blocks, turns one page of book at a time, draws circle visual speech and language - understands prepositions, 3-4words joined together social- interactive play with sharing, eats with fork and spoon, plays without parents
54
what would be expected in each domain in a 4 year old
gross motor - walks up and down stairs as an adult fine motor - draws square/ cross visual speech and language - understands 2 step commands, complex sentences social- sympathy for others if hurt, has a best friend, bladder control, imaginative play, dressing and undressing
55
what would be expected in each domain in a 5 year old
gross motor fine motor - draws triangle , buttons visual speech and language - tells a story using past tense social- handles knife, takes turns, sings/dances/ acts
56
differentials for delay in gross motor
cerebral palsy duchenne muscular dystrophy prader willi?
57
differentials for delay in fine motor/ visual
visual impairment dyspraxia cerebral palsy
58
differentials for delay in speech and language
hearing problems neglect ASD learning difficulties cerebral palsy (multiple)
59
differentials for delay in social domain
neglect ASD
60
differentials for global developmental delay
down syndrome fragile X cerebral palsy
61
red flags in development
no smile at 10 weeks hand preference before 12 months cant sit unsupported at 12 months cant walk at 18 months
62
management of suspected cerebral palsy
MDT approach: paediatrician, neurologist nurse, PT, OT, SALT, dietician, psychology Aiming to improve quality of life management varies from pr to pt PT to help with walking may give some muscle relaxants eg. botulin toxin injections stiffnes- baclofen, diazepam pain management using pain ladder SALT for speech and swallowing OT- identify struggles in every day tasks and find was to optomise anticholinergics for saliva control optomise sleep hygiene, melatonin opthmalologist
63
risk factors for cerebral palsy
prenatal: chorioamnionitis maternal infection congenital infection eg. rubella, toxo, CMV hypoxic brain injury eg. difficult labour traumatic brain injury meningitis
64
classifications of cerebral palsy
spastic CP (70%)- lesion in cerebral cortex causing damage to UMN, reduced GABA causing increased tone and permanent flexion of muscle groups dyskinetic- lesion in the basal ganglia and substantia nigra. issues with initiating and preventing movement- chorea and dystonia ataxic- damage to the cerebellum causing ataxia, discoordination
65
paediatric vital signs ranges (HR and RR)
<1 HR: 110-160 RR: 30-40 1-2 HR: 100-150 RR: 25-35 2-5 HR: 90-140 RR: 25-30 5-12 HR: 80-120 RR: 20-25 >12 HR: 60-100 RR: 15-20
66
what is the definitive management of pyloric stenosis
Ramstedt pyloromyotomy
67
signs of non accidental injury
inconsistent story with injuries/ history keeps changing repeated attendance to A&E (not GP) child looks frightened late presentation physical: - bruises in unusual places - different bruises at different stages of healing - bruising in a non mobile child - failure to thrive
68
management of neonatal sepsis
urgent admission and treatment on suspicion Investigations: - urinanalysis - blood cultures - FBC, CRP, LFTs, U&Es - VBG/ABG -LP Treatment - IV benzylpenicillin and gentamycin - oxygen - IV fluids and electrolyte maintenance - prevension of hypoglycaemia
69
Signs of neonatal sepsis
signs of resp distress: grunting, nasal flaring, recessions, tachpnoea (85%) apnoea (40%) change in mental state, lethargy jaundice (35%) reduced feeding (30%) vomiting (25%) abdo distension (20%)
70
murmur is described as a 'continuous blowing noise' heard below both clavicles.
venous hum
71
causes of snoring in children
obesity downs syndrome tonsilitis nasal problems: polyps, deviated septum, hypertrophic nasal turbinates hypothyroidism
72
dose of dex in croup
0.15mg/kg
73
features of congenital rubella
sensorineural deafness cataracts CHD (PDA) glaucoma Growth retardation Hepatosplenomegaly Purpuric skin lesions 'Salt and pepper' chorioretinitis Microphthalmia Cerebral palsy
74
features of congenital toxoplasmosis
cerebral calcification corioretinitis anaemia hepatosplenomegaly hydrocephalus cerebral palsy
75
features of congenital CMV
microcephaly Low birth weight sensorineural deafness purpuric rash visual impairments anaemia hepatosplenomegaly jaundice encephalitis pneumonitis learning disabilities cerebral palsy
76
what is in the 6-in-1 vaccine and when is it given?
who did the path haematology help? whopping (pertussis) diptheria tetanus polio haemophilius influenzae hepatitis B 2,3 and 4 months
77
whats in the 4-in-1 and whens it given?
who did the path whopping diptheria tetanus polio 3-4years
78
whats in the 3-in-1 and whens it given?
did the path diptheria tetanus polion 13-18years
79
when is MMR given
12 months and booster at 3-4years
80
when is MenB given?
2,4 and 12 months
81
whens the pneumococcal vaccine given?
3 months and 12 months
82
when is menACWY given?
pre uni/ 13-18yrs
83
when is HPV given?
12-13 years to girls and boys
84
what is the difference between Men ABCWY?
N meningitidis is classified into different serogroups based on the components that make up the polysaccharide capsule. The different vaccines therefore encompass different surface antigens specific for the different serogroups there are 6 main serogroups that casue invasive meningococcal disease
85
What are tet spells and what can be a quick method to improve
increase in O2 demand leading to cyanosis. usually happens during feeding or crying bending of legs kinks the femoral arteries and increases vascular resistance which increases BP and increases the pressure in the left side, reversing the VSD shunt
86
murmur heard in tetralogy of fallot
pulmonary outflow obstruction --> ejection systolic murmur heard loudest at left sternal edge
87
1. what is the most common complication of measles? 2. what is the most common cause of death as a complication of measles?
1. otitis media 2. pneumonia
88
cyanotic congenital heart defects
5Ts+H Transposition of the great arteries Tricuspid atresia Tetralogy of fallot Trunctus arteriosus Total anomalous pulmonary venous connection (TAPVC) + hypoplastic left heart syndrome
89
Features of Transposition of the great arteries
cyanosis noticed almost immediately no improvement with saturations loud S2 egg on a string appearance on CXR associated with maternal diabetes
90
management of TGA
1. maintenance of PDA with prostaglandin E2 - NICU/PICU 2. surgical fixation
91
features of tricuspid atresia
valve doesnt form so no blood flow in RV so it doesnt develop (hypoplastic) VSA/ ASD and PDA cyanotic baby noticed in days of birth ejection systolic murmur
92
features of TOF
1. Pulmonary outflow tract obstruction (eg. stenosis) 2. hypertrophy of right ventricle 3. VSD 4. overriding aorta cyanosis noticed in weeks/ months boot shaped heart on CXR harsh sings systolic ejection murumur ans single S2
93
murmur heard in ebsteins anomaly
pansystolic (tricuspid regurg) and mid diastolic (tricuspid stenosis)
94
features of fetal alcohol syndrome
microcephaly (small head) short palpebral fissures (small eye opening) hypoplastic upper lip (thin) absent philtrum reduced IQ variable cardiac abnormalities.
95
what are some signs of DMD?
delayed walking calf pseudohypertrophy Gowers sign (using hands to help stand) 30% have LD later wheelchair respiratory distress from weak diaphragm dilated cardiomyopathy
96
difference between duchenne and becker muscular dystrophy
same dystrophin gene but duchenne is when there is absence of dystrophin and beckers is mishapen dystrophin duchenne- presents by 5yrs beckers- presents by 10-20
97
what is the prognosis of DMD?
most will be fully wheelchair bound by 12 life expectancy of around 25-30
98
criteria for immediate CT after head injury
Loss of consciousness lasting more than 5 minutes (witnessed) Amnesia (antegrade or retrograde) lasting more than 5 minutes Abnormal drowsiness Three or more discrete episodes of vomiting Clinical suspicion of non-accidental injury Post-traumatic seizure but no history of epilepsy GCS less than 14, or for a baby under 1 year GCS (paediatric) less than 15, on assessment in the emergency department Suspicion of open or depressed skull injury or tense fontanelle Any sign of basal skull fracture (haemotympanum, panda' eyes, cerebrospinal fluid leakage from the ear or nose, Battle's sign) Focal neurological deficit If under 1 year, presence of bruise, swelling or laceration of more than 5 cm on the head Dangerous mechanism of injury (high-speed road traffic accident either as pedestrian, cyclist or vehicle occupant, fall from a height of greater than 3 m, high-speed injury from a projectile or an object)
99
list the features of meckels diverticulum (rule of 2)
2% of population 2% symptomatic 2x more common in males 2 years old 2 feet from iliocaecal valve 2 inches long 2/3rds have ectopic tissue 2 types of ectopic - gastric and pancreatic mucosa
100
symptoms of meckels diverticulum
abdominal pain mimicking appendicitis rectal bleeding Meckel's diverticulum is the most common cause of painless massive GI bleeding requiring a transfusion in children between the ages of 1 and 2 years intestinal obstruction secondary to an omphalomesenteric band (most commonly), volvulus and intussusception
101
investigations and management of meckels
abdo USS technitium pertechnetate 99 scan mesenteric angiography Mx may require transfusion if haemodynamically unstable Surgery- wedge excision or bowel resection and anastamosis

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