Pastest - wrong answers Flashcards
S/e of phenytoin
Balance - cerebellar like symps
Mental slowing
Nystagmus
liver dysfunction
How is Caeruloplasmin affected in Wilsons
LOW
Accelerated Hep B vaccination schedule in neonates
Birth
1 month
2 months
12 months
Most common cause of HFMD
Coxsackie A
Then enterovirus
Complications of VZV
Pneumonia
Encephalitis
Cerebellar ataxia
Sepsis
Congenital VZV infection
Limb hypoplasia
Microcephaly
Cataracts
Growth retardation
Skin scarring
What is purpura fulminans & causes of it
Thrombotic disorder with haemorrhagic skin infarction & DIC
Meningococcus
Strep
VZV
Gram -ve bacilli
ECG appearance in rheumatic fever
Prolonged PR interval ie 1st degree HB
Presentation rheumatic fever
2-4w after Group A strep infection
Diagnostic criteria for Rheumatic fever
Jone’s criteria =
Need 2x major or
1x major and 2x minor
Major diagnostic criteria for rheumatic fever
Polyarthritis
Carditis
Sydenham’s chorea
Erythema marginatum
Subcutaneous nodules
Minor diagnostic criteria for rheumatic fever
Fever
Prolonged PR interval on ECG
Raised inflammatory markers
Most common heart problem after rheumatic fever
Mitral valve problems
How long do joint Sx need to be present for diagnosis of JIA
> 6w
Role of diuretics in heart failure secondary to VSD
Help to relieve pulmoanry congestion caused by L>R shunting
‘Dancing eyes, dancing feet’ syndrome
Paraneoplastic syndrome associated with NEUROBLASTOMA
Aka opsoclonus-myoclonus > rapid involuntary horizontal and vertical eye movements
Prognostic sign for opsoclonus-myoclonus in neuroblastoma
GOOD prognostic sign
Appearance of toxoplasmosis on Fundoscopy
White-yellow Chorioretinitis lesions
Sources of toxoplasmosis infection
Contaminated or undercooked meat and dairy products, cat faeces
Role of Hb electrophoresis
Separates subsets of Hb into bands to determine the presence of abnormal subsets e.g. HbS - used to diagnose haemoglobinopathies
What does a gallop rhythm indicate
Sign of acutely impaired ventricular function, resulting in pulmoanry and venous congestion
S/e of carbamazepine
Vit k deficiency
S/e phenytoin
Congenital malformations
Vit k deficiency
Which anti-epileptic is considered safest in pregnancy
Keppra
MOA metoclopramide
Dopamine antagonistw
Why is metoclopramide not liceneced in paeds
Can cause a dystonic reaction
GI complication of CF and how is it caused
Distal intestinal obstruction syndrome - increased level of dehydration and poor pancreatic enzyme compliance > sticky faeces > blocked gut
Asthma stepwise Rx
- SABA prn
- very low dose ICS or LRTA (if < 5 y)
- Very low dose ICS
+ LABA or LRTA (5 years or up)
+ LRTA (< 5y) - Low dose ICS or + LABA or LRTA
Central vs peripheral precious puberty
Central will present in the same order as normal puberty but early
peripheral will present in an abnormal order
What can sometimes be the first presentation of T cell lymphoma
Upper airway obstruction ‘widened mediastinum’
How many mutations needed for a CF diagnosis
2
Level of sweat chloride test for a diagnosis of CF
> 60 mmol/lmos
Most common location for a coarctation
Just distal to the left subclavian artery
Presentation of aortic stenosis
Systolic murmur with ejection click, radiates to the carotids
Presentation of mitral regurgitation
Pan systolic murmur
High-pitched
Loudest of apex
Radiation to left axilla
Rx for shigella
Ciprofloxacin
Rx for giardia
Metronidazole
Rx for severe campylobacter
Azithromycin
Definition latent TB
No clinically active TB
Asymptomatic and not infectious
State of immune response to stimulation by TB antigens
Samples to send to micro for TB diagnosis
Sputum
Gastric aspirates
BAL
Ix for TB
CXR
tuberculin skin tests
Microbiology (3x samples)
Limitation of interferon test for TB
Doesn’t distinguish between active and latent TB so is normally done with tuberculin skin tests
Management of neonatal contact with TB
If no clinical signs of TB
Then give
Isoniazid prophylaxis
And do tuberculin skin test at 3 MONTHS
If TST -ve, then repeat with interferon text and if this is negative then stop Abx
Bacterial cause of toxic shock syndrome
Toxin producing strains of S. aureus or Group A strep (strep pyogenes)
‘Super antigens’ that can non-specifically bind to T-cell receptors and cause increased inflammatory response
Presentation toxic shock syndrome
Red palms & soles with desquamation
‘Sand-paper’ maculopapular rash
High fever
Vomiting diarrhoea
Mucositis
Myalgia (high high CK)
Adult Hb
Alpha and beta
Rx salicylate poisoning
Urinary alkalinisation : 1L NaBic over 2hc
Impetigo - school rules
Keep off until affected areas are crusted and healed OR 48h after starting Abx
Why can low Ca cause stridor
Laryngeal collapse due to loss of ridigity
What is a laryngeal web
The larynx contains web-like tissue that partially constricts the trachea and causes a CHRONIC stridor
Inheritance of epidermolysis bullosa simplex and layer of skin affected
AD
Within the epidermis
Inheritance of junctional epidermolysis bullosa
AP
Lamina Lucida of the basement membrane
Inheritance of dystrophic epidermolysis bullosa
AD (mild), AR (severe)
Uppermost dermis
Affected arches in DiGeorge
3rd and 4th pharyngeal arches
Which is more severe and how do the presentations differ between ABO incompatibility and Rh disease
ABO incompatibility generally milder disease than Rh
Liver and spleen not normally enlarged in ABO but are in RhD
Presentation of tinea rash
Annular erythema with central clearing
IX dermatomyositis
Muscle biopsy
MRI
Type of anaemia in hypothyroidism
Macrocytic
How unwell are patients with IgA deficiency
Recurrent infections but well in between
No faltering growth
Usually found incidentally, can have no clinical Sx
What is Bruton’s disease
Aka X-linked agammaglobulinaemia
Presents as a male with recurrent infections and faltering growthw
What is sprengel’s shoulder
Failure of the scapula to descend secondary to fibrosis
Renal conditions associated with Turners
Horseshoe kidney
Renal agenesis
Coagulation problem associated with Noonan’s
Factor XI deficiency
Causes of acute interstitial nephritis
Drugs (cipro, erythromycin, phenytoin, PPIs, NSAIDS)
Infection
Ix for acute interstitial nephritis
Urinary eosinophils raised
Red cell casts
Hyperkalaemia
Metabolic acidosis
AIN - type of hypersensitivity
Type IV
Presentation carbamazepine toxicity
Cerebellar Sx
Triad for HUS diagnosis
Microangiopathic haemolytic anaemia with red cell casts
Thrombocytopenia
AKI
Why are patients with sickle cell more likely to get sepsis
Repeated splenic infarcts causes splenic malfunction
Rx tinea capitis
SYSTEMIC griseofulvin
(Topical isn’t effective in penetrating hair follicles)
What is Wiskott-Aldrich syndrome
A primary immunodeficiency
Affects B and T cell lymphocytesWha
Live vaccines - allowed or not allowed in Wiskott-Aldrich syndrome
Contraindicated !
Is a cholera vaccine routine
No, normally only for emergency aid workers
Duplicated ureter - how to know if the insertion of the second ureter enters the normal position
Continence WONT be affected if the ureter enters in the normal position
If it enters into an abnormal position then the patient will be incontinent
What is cystinosis and inheritance
AR
Lysosomal storage disorder
Blood gas in cystinosis
Hyperchloraemic
Hypokalaemia
Metabolic acidosis
Normal anion gap
Surgical treatment for coarctation
Balloon angioplasty & stenting
Surgical treatment for aortic stenosis
Balloon valvuloplasty
Surgical treatment for TGA
Arterial switch
Definition of odds ratio
Measure of the strength between an exposure and an outcome
what always needs to be taken into account when calculating odds ratio
Confounders
Ie a factor that can influence the strength of the association, causing a false high or low
Congenital rubella presentation
Cataracts
Deafness
Retinopathy
CHD
Hepatosplenomegaly
What causes acanthosis nigricans
Overgrowth of epithelial cells secondary to increased insulin production
Mutation ataxia telangiectasia
ATM gene on chromosome 11q26
Presentation ataxia telangiectasia
Progressive neurology - tremor, chorea, athetosis, dystonia, ataxia, dysphasia
Telangiectasia
Presentation benign Rolandic epilepsy
School age boys
Nocturnal partial seizures
Speech arrest
Secondary generalised seizure
Which antiepileptic NOT to use in myoclonic seizure
Lamotrigine
Tetralogy of Fallot
VSD
Overriding aorta
RVH
RV outflow obstruction (pulmonary stenosis)
Effect of Mg on PTH
A low Mg inhibits PTH release
Effect on Alk P when low Ca is secondary to hypoparathyroidism
Normal
Effect on Alk P when low Ca is secondary to Vit D disorder
High
What is wrong in Pseudohypoparathyroidism
Failure of the action of PTH (genetic problem)
Blood abnormality in extra hepatic biliary atresis
Thrombocytosis
Definition of high conjugated fraction
> 200 or > 20% of total
Presentation of PCD
Recurrent chest, sinus and middle ear infections
Situs inversus, Kartagener syndrome
S/E of vincristine
CENTRAL neurotoxicity
- headache, malaise, dizziness, seizures, depression
PERIPHERAL neurotoxicity
- loss of DTR, wrist/foot drop i.e HIGH STEPPING gait
AUTONOMIC neurotoxicity
- hoarseness, ptosis, strabismus
Inheritance of SMith-Lemli-Opitz syndrome and how it presents
AR
Cholesterol synthesis disorder
Midline CNS problems
Syndactyly
Microcephaly
Low set ears
Ptosis
Micrognathia
IM adrenaline dose <6m
100-150 mcg
(0.15mL)
IM adrenaline dose 6m - 6y
150 mcg (0.15ml)
IM adrenaline dose 6y-12y
300 mcg (0.3mL)
IM adrenaline dose >12 y
500 mcg (0.5mL)
Drugs causing peripheral neuropathy
Amiodarone
Cisplatin
Ethambutol
Isoniazid
Metronidazole
Nitrofurantoin
Phenytoin
Vincristine
Landau-Kleffner syndrome
Seizure disorder and expressive or receptive aphasia
Normal hearing
EEG in Landau-Kleffer syndrome
Bitemporal abnormalities
Melanosis coli on colonoscopy - indicative of?
Senna abuse
Inheritance of Tay-sachs and what is it
AR
Lipoidosis due to deficiency of the enzyme hexosaminidase
What happens in SIADH - explain the blood tests
Water is retained inappropriately by the kidney
Therefore urine osmolality is inappropriately high in relation to plasma osmolality
Low volume but high NA containing urine
X-ray of osteosarcoma
Intramedullary sclerosis
‘Sunburst’ periosteal reaction
Definition of toxic megacolon
Dilated >6cm transverse colon and at least 3 of:
Fever >38
HR >120
Neutrophilia
Hb < 105
SUFE Rx
Pin & screw
To preserve blood supply and prevent AVN
Grade 1 VUR
Reflux of urine in to the ureter ONLY on micturition
Grade 2 VUR
Reflux into the ureter, pelvis & calyces with NO DILATATION on micturition
Grade 3 VUR
Reflux of urine into the pelvis & calcyces with MILD DILATATION
Grade 4 VUR
MODERATE DILATATION of the ureter, pelvis and obliteration of the sharp angle of fornices on micturitionF
Grade 5 VUR
GROSS DILATATION and tortuosity with no papillary impression visible in calyces
Definition of PUV
Mucosal folds in the posterior urethra of infant boys
Most common UNILATERAL
L > R
What is Fabry’s disease and how it presetns
X linked recessive lysosomal storage disorder
Stroke, skin lesions, renal insufficiency, heart attacks
Presentation Homocystinuria
Marfanoid habitus
Strokes
Intellectual disability
Lens abnormality
Asplenia is a contraindication to live vaccination T. Or F
False
Rubella presentation
Fever with rash on Day 1/2
Lymphadenopathy in posterior auricular or occipital region
Measles presentation
Fever with rash on Day 3/4
Koplik spots
Rash progresses from behind the ears
1st line treatment for juvenile myoclonic epilepsy
Sodium valproate
Keppra
Long QT - how to tell apart Jervell-Lange Nielsen syndrome and Romano-Ward syndrome
JLNS - AR, associated with congenital deafness
RW - AD, not associated with deafness
Medications causing prolonged QT
Macrolides
Antimalarials
Antipsychotics
Definition of Brugada syndrome
Specific ECG pattern : ST elevation in R precordial leads V1-V3 followed by inverted T wave
+ 1 of
: VF, polymorphic VT, FHx of sudden death
Diagnostic criteria for infective endocarditis
2x major OR
1x major and 3 minor OR
5x minor
ECG appearance of pulmoanry stenosis
Prominent R waves and upright T wave in V1
Alagille syndrome is associated with what heart abnormality
Pulmonary stenosis
Dose of adenosine
0.1mg/kg
What is Ebsteins anomaly
Inferiorly displaced tricuspid valve, so there is a smaller RV
question recognising this on MRI
Definition of hypertension
Systolic and/or diastolic BP > 95th C for age & sex measured on 3 separate occasions
1st line / 2nd line Rx for HTN
- CCB - nifedipine
- BBs
What condition are CCBs contraindicated in
Diabetes
Radiation of murmur in mitral regurgitation
Axilla
How does adenosine work
Slows conduction through the AV node
VT Rx if conscious vs unconscious
conscious - amiodarone
Unconscious - 1J/kg synchronised DC shock
Complication of coarctation surgery
Ischaemia of the spinal cord due to prolonged cross-clamping of the aorta
Pressure graph of the heart chambers and the PA and aorta
RA: 2-8 LA: 2-10
RV: 15-30/2-8 LV 100-140/3-12
PA: 15-30/4-12 Aorta: 100-140/60-90
What is the aim of a Tet spell treatment
Increase pulmonary blood flow by reducing PVR
- morphine, O2, correction of metabolic acidosis
Reduce L>R shunting by increasing SVR
- knees to chest, volume administration, vasoconstrictors
Digoxin toxicity on ECG
Prolonged PR, ‘reversed tick’, ST depression
Cardiac problems seen in Williams syndrome
Peripheral pulmonary stenosis
Supravalvular aortic stenosis
Indications for early surgical closure of VSD
Presence of HF
Faltering growth
Signs of pulmonary hypertension
When should children with diabetes get renal screening and what is measured
From diagnosis annually
Protein/Creatine ratio
What are insulin levels like in T2DM and why
Usually raised
Due to insulin insensitivity
T1DM fluid regime during surgery
0.9% NaCl + 5% dex + 20mmol KcL + insulin
Criteria for T1DM diagnosis
Random venous plasma glucose 11.1mmol or greater OR
Fasting glucose 7 or greater OR
2h glucose conc 11.1 or greater 2h after 75g glucose ingestion
Target BMs in T1DM
4-7 on wakening and before meals
5-9 after meals
How to calculate total daily dose of insulin
Weight (kg) = 0.75 units insulin
How to calculate insulin sensitivity
100 / total daily dose of insulin
What % of insulin in T1DM is long acting and short acting
Long acting = 40% of TDD
Short acting (60% of TDD)
Dose regime for calculating long-acting insulin requirement
100 x 0.75 x 0.4
Dose regime for calculating short acting insulin requirement
100 x 0.75 x 0.6
Then divide this into 3 for meal time doses
Rx cerebral oedema in T1DM
30 degrees head up
Induce an osmotic diuresis with IV 20% mannitol 0.5-1g/kg over 10 mins OR
hypertonic saline (3%) 5ml/kg over 10 mins
In diabetes insipidus, what will happen to urine osmolality after water deprivation
It will remain inappropriately low (<300 mosmol/kg)
Urine osmolality after desmopressin in cranial DI
It will increase (because you’ve replaced the ADH)
> 800mosmol/kg
Urine osmolality after desmopressin in nephrogenic DI
It will stay low (because the kidney can’t action the ADH) so can’t retain urine
< 300mosmol/kg
Causes of nephrogenic DI
Congenital
Hypercalcaemia
Hypokalaemia
Drugs (lithium, amphotericin B)
Kidney disease
Sickle cell
Sjogren’s
Mid-parental height calculation
(Fathers + mothers height - 13) / 2
How to know the difference between exogenous and endogenous insulin
Endogenous - the precursor has a c-peptide attached which is removed to make active insulin
Exogenous - only the active form, no precursor
Tumours associated with MEN1
Pituitary adenoma
Parathyroid hyperplasia
Pancreatic tumours
Tumours associated with MEN2A
Parathyroid hyperplasia
Medullary thyroid carcinoma
Phaeochromocytoma
Tumours associated with MEN2B
Mucosal neurons
Marfanoid body habitus
Medullary thyroid cancer
Phaeochromocytoma
Non-toxic goitre - normal or abnormal TFTs
Normal
Normal thymus on CXR
‘Sail shape’
Rx status epilepticus
IV access - 1. Lorazepam
Then repeat if required
Then Keppra
Percussion of tension pneumothorax
Hyperresonant on same side
1st degree burn
Epidermis only
No blistering
2nd degree burn
Epidermis and the papillary and reticular layers of dermis
Blisters
Painful
3rd degree burn
Full thickness epidermis & dermis
Not painful
What test should all be done in fire victims
Carboxyhaemoglobin
Burns fluid resus
4ml of ringers lactate / kg per % BSA
Give 1/2 in the first 8h, starting from TIME OF INJURY, then the other half over 16h
TCA OD on ECG
Prolonged QTc
Widened QRS
Which predisposes to VF or VT
What to do in extravasation injury
Aspirate the cannula
Don’t de roof blisters
What grade does an extravastion injury start blanching
Grade 3
How to differentiate Grade 3 and 4 extravasation
Grade 3 - normal pulses and CRT
Grade 4 - prolonged, possible absent pulses
Definition of sepsis
Hypoperfusion, hypotension, and organ dysfunction
Predictors for severity of liver injury in paracetamol injury
: arterial pH < 7.3 and lactate > 3.5 after fluid resus
: creatinine > 300
: INR > 6.5
: elevated PT
: grade III or grade IV hepatic encephalopathy
GCS
E4
: spontaneous
: to voice
: to pain
: none
V5
: orientated
: confused
: incomprehensible words
: incomprehensible sounds
: none
M6
: obeys commands
: localises pain
: normal flexion to pain
: decorticate
: decerebrate
: none
management of choking ep
Check if cough is effective
If it is then encourage coughing and check for deterioration
Keppra dosing in status
40mg/kg over 5 mins