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672 Cards in this Set

  • Front
  • Back
Which level of skin differentiates between superficial burn and deep burn?
mid dermal level
What does a superficial burn look like?
What layer of skin is affected?
sunburn (pale erythema)

epidermis
What layer of skin is burnt if it's dark blotchy erythema?
Does it blanch?
deep dermis

no blanching
Burn blisters: deep or superficial burn?
Will it have a blanching base?
Superficial
Yes - the blanching means there is blood flow so it can recover
Why do you get dehydrated when burned?
burn injury is vasomodulating --> vasodilation --> protein pours through --> loss of volume from circulation --> local oedema and general hypovolaemia
the fluid carries albumin
What are the two most important points for first aid for burns?
1. stop the burn
e.g remove hot clothing, stop drop roll, turn off current, brush chemical off
2. cool the burned surface
Use flowing water (reduces depth of burn)
What do you hope to achieve by putting running water on a burn?
What temp should the water be?

Start water ASAP but if you can't how long after can you start?
flowing water reduces depth of burn
the zone of stasis no longer --> zone of necrosis

8-25 deg (causes vasconstriction)

3h
How long do you run water onto burn for?
at least 20mins
Respiratory burn:
Where in the airways will you get thermal injury?
supraglottic
--> early obstruction
Respiratory burn:
Where in the airways will you get injured from chemicals?
infraglottic
--> pneumonitis
Early assessment of burns is inaccurate/
How long do burns change depth for?
48h
Fluid resus in burns
Formula
What is this formula called?
What solution is used?
How is it given?
Parkland's Formula

4mL/kg/%BSA in first 24h
Hartmann's

Give half in first 8h after got the burn
Give the 2nd half over the next 16h
When would you add maintenance fluids to burns resus fluids?
child <30kg
Do you give fluid resus to every child who's sustained a burning injury?
No, only if over 12% of BSA is burnt
What should urine output be in kids?
UO: 1mL/kg/hr

Increase fluids if UO too low, and decrease if UO too high
After the first day, what do you wash the burn with?
Chlorehexidine
Should you drain and/or deroof burn blisters?
Yes
What prophylactic vaccine do you have to give to burns' patients?

Do you need to give ABx?
tetanus

no
How often should burns dressings be changed?
What do you have to remember when doing this?
at least once per day
PAIN RELIEF
What sort of surgery is gold standard for burns?
Split skin autograft
How do you estimate BSA burned?
Rule of 9s

Area of hand = 1%
Head of infant = 18% (for every yr up til age 10, take 1% off heads and add to leg)
Leg child = 14%
Arm = 9%
Chest = 18%
Back = 18%
When would you admit a burn? (3)
if airway affected
face and peripheries, joints
hands and feet (need special care)
When would you transfer a burn? (6)
1. any child burn >5% TBSA
2. resp burn
3. burns of special areas
4. suspicion of child abuse
5. concomitant trauma
6. pre-existing sig disease
If a burn heals between ___days and ___days then it won't scar
10-14
If a burn heals between ___weeks and ___weeks then it can be neglected and won't contract over the joint.
2-3

these need to be followed up
If a burn heals >___weeks will definitely scar. What is the mgmt?
graft
follow-up
Mgmt of burns over joints?
splint
Dx:
jerks last 10-20s when child asleep
10-20s duration
stop abruptly when child awakened

What is the onset?
When should it remit?
Benign Neonatal Sleep Myoclonus

d1 - 3wks

remits bu 2-7mo
What sort of Sz can you get from holding your breath?
hypoxic induced Sz

(this is not epilepsy)
Dx:
asynchronous tonic or clonic movements, most of face, neck, extremities
Some have aura
10-20s
Eye deviation (can lateralise)
Can be responsive
No postictal period
EEG: spike and sharp waves or multifocal spikes
Simple partial sz
(aka simple Focal)
Dx:
impaired consciousness
can respond during episode
some have aura
automatisms
EEG: sharp waves or focal spikes

What sort is it if they put their hand towards their throat as a warning?
Complex partial sz
(aka complex focal)

Temporal
Dx:
sudden cessation of motor activity or speech with blank stare and flickering eyes
No aura
<20s
No postictal period, but child can't remember it
EEG: 3/second spike and generalised wave d/c (3Hz)
Generalised Absence Sz
(Petit Mal)
Dx:
May have aura
LOC
Eyes roll back, tonic contraction, apnoea
Then clonic rhythmic contractions
Tongue biting, loss of bladder ctl
Postictal: semicomatose 2h, vomiting and H/A
Generalised Tonic/Clonic Sz

(NB the aura indicates localisation - in this circumstance it can say where the Sz originated)
Dx:
Symmetric contractions of neck, trunk and extremities - looks like Moro reflex w/o stimulus
Multiple episodes per day
Occurs around 6mo
Assoc with regression in skills
EEG: hypsarrhythmia

What is another term for this Sz type?
Infantile Spasms (AKA West Syndrome)

Jack Knife
What is the cause and Rx for Infantile Spasms?
increased corticotropin-releasing factor -> neuronal hyperexcitability

Rx: ACTH or steroids
Dx:
brief, symmetric muscle contraction, typically seen in upper arm first thing in morning
loss of body tone and falling forward
Presents as teenager
Grow out of it 15-20yrs
Juvenile Myoclonic Sz
Dx:
Sz that typically occurs overnight
often hypermotor; v. agitated, behave unusually
v. brief (~30s)
Frontal lobe Sz
What do you think of with high pitched scream and bulging fontanelle?
raised ICP
Bile stained vomit = ______________ until proven otherwise
bowel obstruction
When assessing a child's airway, what does drooling mean?
What should you do?
airway difficulty

get help!
Single most important way to assess circulation?

3 places where can do it

What is normal?
Capillary refill

1. sternum
2. forehead
3. base of foot

<2sec = N
What's the acronym for assessing disability?
What does it stand for?
Which one corresponds to the GCS that requires intubation? What GCS is this?
AVPU
A = alert
V = only responds to verbal stimuli
P = only responds to painful stimuli (= GCS 8 --intubate)
U = unresponsive
What does decorticate mean (GCS)?
Decerebrate?
flexed limbs

extended limbs
Less than what of normal fluid intake indicates reduced intake?

Reduced urine output = <____ wet nappies in past 24h
< half

< 4 nappies
What is the acronym for assessing the deteriorating child?
A - airway
B - breathing
C - circulation
D - disability (AVPU)
E - exposure
F - fluids
G - glucose
Hypoglycaemia

Normal BGL = ______-_____ mM

BGL ____-____mM needs review
BGL < 2 requires response promptly
3-5

2-3

2
Rx for hypoglycaemia
2mL/kg of 5% dextrose
5 x DDx for upper airways emergencies
1. croup
2. epiglottitis
3. tracheitis
4. retropharyngeal abscess
5. FB
What organisms (2) cause trachietis?
Staph
Strep
What part of the airway is affected in croup?
subglottic (from cords down)
DDx for lower airway emergencies? (4)
1. bronchiolitis
2. asthma
3. pneumonia
4. FB
Most significant clinical sign in asthma?
pulsus paradoxus
- normally when inspire, pulse drops as more and more thoracic obstruction, when expire, pulse increases
does opposite in PP
What do you see on CXR in asthma?
overinflation
Define fever
>38 degrees
What are the causes of fever? (hint: mneumonic)
SOURCE

Skin
Osteomyelitis
UTI
Resp: bronchiolitis (+OM, tonsillitis)
CNS
Enteric
Septic workup (7)
1. FBC
2. blood film
3. blood culture
4. CXR
5. UA
6. stool culture/faecal WCC
7. LP
Red flags for vomiting (5)
1. bilious
2. fever >39
3. blood
4. severe abdo pain
5. very young (hydrocephalus...think congenital)
Two pathways that kids --> cardiac arrest
Circulatory failure (fluid loss/maldistribution)
Respiratory failure (resp distress/depression)
In infants, you worry that deterioration is imminent if RR > ____
60 (normal for <1yr = 30-40)
Classic red flags (7)

Resuscitate, assess and treat without delay
1. purpuric rash
2. bulging fontanelle
3. biphasic stridor
4. high pitched scream
5. bile stained vomit
6. persistent tachy
7. grunting respiration
Initial mgmt of the deteriorating child?
Assess: arousal, alertness, activity, breathing, colour
ABCDEFG
Give O2
Position child
Call for help
Insert IV
What are the signs for increased work of breathing? (6)
tachypnoea
and
1. recession
2. grunting
3. nasal flair
4. accessory m use
5. head bobbing
6. cyanosis
What are the effects of inadequate breathing (3)?
heart rate increases
skin colour: pallor/cyanosis
mental status: agitation/exhaustion/drowsiness
What are the S+S of hypoglycaemia (2 groups)
Autonomic: trembling/jittery, pounding heart, cold sweat, pallor

Neurologic: slurred speech, confusion, irritable, erratic, LOC/Sz
Decorticate posturing suggests a lesion ______ the brainstem

Decerebrate posturing suggests a lesion ______the brainstem
above


below (midbrain or pontine)
How do you classify croup as being mod/severe instead of mild?
stridor at rest
Rx of severe croup (3) (incl doses)
ADMIT

1. O2
2. dexamethasone 0.15mg/kg
3. SDNE (neb Ad) 4mL of 1:1000
Rx for bronchiolitis
Keep SaO2 >94%
fluids: mild (RR<60) - PO; IV if severe distress
Trial of neb salbutamol 2.5mg or atrovent but if doesn't help, stop
Do you give ABx or steroids in bronchiolitis?
No
What level of PaO2 would you need before intubating?
<50mmHg
Pulse oximetry <____% in severe asthma
93
Nebulised salbutamol dose in severe asthma attack
2.5-5mg/4mL with 8L/min O2 up to three times in first hour
Dose Prednisone / Hydrocortisone in severe asthma attach
Pred: 1-2mg/kg PO

Hydrocortisone: 5mg/kg IV
Dose IV salbutamol
0.5-15ug/mg/min
Approach to febrile infant:
Is the child toxic? What 5 signs of being toxic?
1. lethargy
2. poor feeding
3. irritability
4. poor perfusion
5. reduced UO
What is the majority cause (90+%) of febrile episodes?
viral illness
Dx:
non-bilious vomiting
ravenously hungry post-vomits
weight loss
reverse peristalsis
hypochloraemic
hypokalaemic alkalosis
Pyloric stenosis
Biochem changes in pyloric stenosis? (ie vomiting) (3)
hypocholoraemia
hypokalaemia
alkalosis
Initial mgmt of Sz (afebrile, <15min) (3)
1. airway protection
2. semi-prone positioning
3. paracetamol

Doesn't yet require Ix
What do you need to rule out if first Sz (afebrile) is >15mins, recurrent, focal, difficult to control fever, new neurological abnormality (6)
1. head injury
2. meningitis
3. drugs
4. encephalitis
5. hypoglycaemia
6. SOL
Febrile Sz: suspect ________
Febrile Sz + altered mental state: suspect ________
meningitis
encephalitis
If a Pt has a Sz are is found to be hypoglycaemic, what is the Rx?
10% dextrose 5mL/kg IV
OR
glucagon 0.5mg (<5yr) IM/IV or 1mg (>5yr)
then glucose IV or CHO feed (if conscious)
Emergency Rx of Sz (5)
1. clear airway, lie on side, give O2
2. check for hypoglyaemia
3. Diazepam 0.2mg/kg IV.
Repeat PRN
OR 0.5mg PR
4. Phenytoin 20mg/kg or phenobarbitone 20mg/kg
5. consider Thiopentone/muscle relaxation and intubation +/- midazolam infusion
What usually precedes mesenteric adenitis?
viral URTI
--> get inflammation of Peyer's Patches near ileocaecal junction
= self limiting disease
Usual age for appendicitis?
6-9
Usual age for intussusception?
<1
What Ix for suspected appendicitis?
What do you expect to see? (5)
FBC - WCC and differential (PMNs > 75%)
CRP
UA (UTI can mimin appendix)
U/S is not sensitive and not specific
U/S is useful in girls if thinking ovarian cause
Mgmt appendicitis (5)
1. Fluid resus 20mL/kg bolus saline + maintenance
2. Pain mgmt
3. explain to parents
4. ABx (metronidazole [covers anaerobic] + gentamycin OR cephazolin [covers aerobic])
5. laparoscopic surgery
What ABx do you give when doing appendicectomy?
Metronidazole (nitroimidazole)
plus
Cephazolin (cephalosporin)
or
Gentamycin (aminoglycoside)
How does malrotation present?
bilious vomiting
abdo pain
How does pyloric stenosis present?
non-bilious vomiting
What shape does malrotation look like when put dye down NG tube and do AXR?
backwards S

S is for surgery!
What do you think of with redcurrent-jelly like stools?
Intussusception
What do you think of with sausage shaped mass, tender in epigastrium or RUQ?
Intussusception
What does intussusception look like on U/S?
target/donut sign
Mgmt of intussusception? (3)
Fluid resus
Pain mgmt
Send to radiology for air enema
Vomiting from pyloric stenosis loses Na, K, HCl and water.
When should you add K to fluids?
after passes urine
Major congenital malformations occur in ~___% of children within the first 5 years of life
5
"Morphological defect of specific anatomical structure occurring DURING organogenesis" = _______________
malformation
"abnormal shape/position of body part due to anomalous mechanical forces*. Usually occurs AFTER organogenesis" = >______________

*e.g co-twin, uterine fibroids, oligohydramnios
Deformation

- loss of symmetry, distorted configuration
"result of a destructive process that alters structures AFTER normal foetal devpt" = _____________
Disruption

e.g amniotic bands --> amputation of parts
Increased ____________ age --> higher risk for new dominant mutations

Increased ___________ age --> higher risk for chromosomal abnormalities
father's


mother's
Any growth parameter measurements that deviate >____ standard deviations from each other are outside the normal range and need an explanation
2
Where is the deletion in Velocardiofacial syndrome?
Chromosome 22q11
What are the facial features of velocardiofacial syndrome? (3)
1. high broad nasal bridge
2. alae nasi underdeveloped giving appearance of a tubular nose
3. small ears that don't have lower lobe
What develops abnormally in velocardiofacial syndrome?
pharyngeal arches
If you suspect, clinically, a genetic condition due to a very large deletion, what specialised testing can you do?
FISH
karyotype
microarray
Incidence of childhood cancer
1 in 600
True or false: kids get carcinoma
False
Adults do
What sort of cancer Rx are paediatric cancers highly sensitive to?
chemo
What percentage of kids with cancer are cured permanently?
75%
What is the most common of all childhood malignancies?
ALL

(followed by CNS tumours)
Presenting symptoms of ALL (9)
1. anaemia
2. bruising
3. fever
4. bone pain
5. adenopathy
6. hepatomegaly
7. splenomegaly
8. malaise
9. anorexia
Which cells do you see in the blood which normally should only be in the BM in ALL?
lymphoid blast cells
ALL:
Which has better prognosis:
Lower WCC or Higher WCC
low
ALL:
Which has better prognosis:
B cell
or
T cell disease
B
How long do you have to have chemo for ALL?
1 month induction
5-mo 'heavy' chemo
1.5yrs 'gentle' maintenanco chemo PO
Which is more serious: AML or ALL?
AML
similar presentation to ALL
different BM aspirate
Where are paeds brain tumours usually located?
posterior fossa

infratentorial
Where would a medulloblastoma be located?
4th ventricle
What does an ependymoma arise from?
ependymal lining of ventricles
Optic nerve glioma is associated with what other condition?
nuerofibromatosis-1
Where is the classic site for a benign astrocytoma?
cerebellum
What are the risks of treating brain tumours in kids? (3)
1. may affect neuro-cognitive fn
2. may cause hypopituitarisn (GH, pubertal derangement, thyroid)
3. carcinogenic (may cause 2ndary cancers)
Where would you usually find a neuroblastoma?

What cells does it arise from?

What rises in the urine?
= tumour of adrenal gland

neural crest cells

HVA
DDx for mass in flank (3)
1. neuroblastoma
2. Wilms' tumour
3. hydronephrosis
What organ would you find a Wilms' tumour in?
kidney

(AKA nephroblastoma)
Are Wilm's tumours and neuroblastomas benign or malignant?
malignant
How does Wilm's tumour present? (2)
1. PAINLESS abdo mass
2. haematuria
Which age group gets Wilms' tumours?
young
(1-7)
What are 3 associated conditions of Wilms' tumour?
1. aniridia
2. hemihypertrophy (half of body grows more)
3. GU anomalies
Which age group gets Hodgkin's disease?
teenagers
What is the diagnostic hallmark cell in Hodgkin's?
Reed-Sternberg cell
How does Hodgkin's present? (2)
1. mediastinal mass
2. supraclavicular/cervical painless, firm LNs

+ fever, weight loss, sweats etc.... PRURITIS
What are three types of Non-Hodgkin's Lymphoma and how do they present?
1. T-lymphoblastic: older, mediastinal mass, pleural effusion
2. Burkitt's (B cell): abdo disease, intussusception, mass
3. large cell NHL
Which virus is associated with Burkitt lymphoma?
EBV
Who typically gets osteosarcoma?
Why?
adolescents

assoc with rapid bone growth
Where in the bones does Ewing sarcoma affect?
diaphyseal long bones
Where in the bonds does osteosarcoma affect?
growing ends of long bones
What is the most common soft tissue sarcomas?
What are the three most common places it's found?
rhabdomyosarcoma

1. head and neck
2. GU tract
3. extremities
Which ABx do you give a child who's having chemo and has febrile neutropaenia? (2)
Timentum
Gentamycin

(need gram +, - and pseudomonas cover)
Over what days does the rostral neuropore close?
d23-26
Over what days does the caudal neuropore close?
d26-30
What is the incidence of paediatric food allergy?

What is the prevalence of paediatric food allergy?

For <5 year olds?
1-3%

1 in 20

1 in 5
Immunological mechanism of IgE mediated allergy
allergen -- processed by macrophage -- presented to T cell --- communicates with B cell --- under Th2 type conditions, B cell switched from IgM/IgG production to IgE production -- circulating IgE binds to mast cells/basophils

re-exposure to allergen -- IgE bound to mast cells -- recognises allergen -- cross linking of IgE -- mast cell/basophil degranulation -- release of histamine
IgE mediated food allergy manisfests itself within how many hours of food ingestion?
2
Define mild IgE mediated food rxn
localised facial erythema or urticaria/angioedema (e.g swollen lips)
Define moderate IgE mediated food rxn
Generalised urticaria/angioedema
or vomiting or both
Define severe IgE mediated food rxn
Generalised urticaria/angioedema
and stridor or wheeze
and/or collapse

Pale/floppy in small infants
If your sibling is atopic, how much does your risk increase of being atopic?

And atopic parent?
4x

10x
You can be allergic to any foods that contain a _________
protein
What are the 8 foods taht cause more than 90% of IgE mediated allergic rxns in kids?
1. eggs
2. milk
3. peanuts
4. tree nuts and seeds
5. fish
6. shellfish
7. soy
8. wheat
Skin prick test:

NEGATIVE if welt <______
What would this tell you?

Why would you get a welt in a positive test?
2-3mm
A negative test means it's highly unlikely you have an allergy to that substance (i.e good NPV)

pre-formed IgE
____% have +ve RAST or skin prick test but AREN'T clinically allergic
50
How does the RAST test work?
have the allergen bound to the test tube
put patient's serum in
IgE will bind to allergen
put detection anti-IgE Abs in that bind to patient's IgE
wash patient's serum away
detect amount of patient's IgE
What is skin prick test good for?
monitoring if grow out of allergy
Can you use skin prick or RAST to figure out severity of allergy?
no
What's a +ve skin prick test?
greater than or = 3mm
Skin prick tests have a fair PPV with combined with ______________ ___________
positive Hx
of symptoms which occur as a result of exposure to the allergen
What does RAST stand for?
radioallergosorbent test
RAST has good PPV at high levels for which 3 things?

What does this mean?
Can you tell severity or likelihood of anaphylaxis from RAST?
egg, milk, peanut

if number is high on the interational units for RAST, it is likely you have the allergy

No
Which is better for ruling out an allergy, RAST or skin prick?
skin prick (has better NPV)
What do you do if your skin test is +ve but you don't know about exposure Hx? OR if you've got a grey area score for RAST

Can you tell severity of allergy from this?
Open Food Challenge

No
Which 4 things which induce allergies are more likely to be outgrown?
1. milk
2. egg
3. soy
4. wheat
How do you give the Epipen?

Doses?
"blue to the sky, orange to the thigh"

Doses: 150 for younger kids
300 if over 20kg
What are the major risk factors for food anaphylaxis in food allergic children? (4)
1. older child
2. increased dose of allergen
3. presence of asthma
4. nature of allergen (nuts, shellfish, fish)
What are the three foods that are more likely to cause anaphylaxis?
What is #1?
nuts (peanuts = #1)
shellfish
fish
How soon after food ingestions do non-IgE mediated food allergies manifest?
2-24h
What is the best test for non-IgE mediated food allergies?
food elimination and rechallenge
Is eosinophilic oesophagitis IgE mediated or non-IgE mediated?
non-IgE mediated
(ie presumed T cell mediated)
In Bx for eosinophilic oesophagitis, how many eosinophils do you see per HPF?
>20
Rx: eosinophilic oesophagitis? (2)
1. dietary modification
2. swallow fluticasone
What does FPIES stand for?

What usually causes it (3)
Food protein induced enterocolitis syndrome

Rice
Soy
Chicken
Do allergic rxns typically get worse over time with more exposure?
no

the rxns are unpredictable
Are skin prick tests good for picking up non-IgE mediated food allergy?
No
Mechanism for T cell mediated allergy

What is this type of rxn called?
allergen processed by macrophage -- presented to T cells -- Th2 environment -- allergen specific memory T cell created

Allergen re-exposure -- allergen processed by macrophage -- Ag presented to memory T cells -- activated T cell proliferation and cytokine production

Delayed type hypersensitivity rxn (Type IV hypersensitivity rxn)
What is the allergic march?

Why is it thought to happen?
progression of atopic disease from:
eczema --> asthma --> allergic rhinoconjunctivitis

caused by a regional allergic response with breakdown of the local epithelial barrier that initiates systemic allergic inflammation
From how old can you do creatinine levels in a neonate?
Why?

What is typical creatinine for a neonate?
from day 3

before that it reflects the mother's

15-30
From how old can you do U/S in a neonate?
Why?
from day 5
there's not enough urine being made to show up on U/S before this, so if there's an obstuction, you wouldn't see it
When do you start toilet training?
2-3 years
How may kids wet the bed?
10% of 5 year olds

5% of 10 year olds
What is normal GFR for a neonate?
30-40
What 4 anomalies can renal U/S show?
1. size
2. cysts
3. dilatation
4. stones
What is MCUG/VCUG?
How does it work?
What 3 things can you Dx with it?
Micturating Cystourethrogram

Catheter -- contrast into bladder -- Pt voids

1. vesico-ureteric reflux
2. posterior urethral valves
3. bladder diverticulum
What do you see on MCUG in vesico-ureteric reflux?
dilated renal pelvis/calyces
(depends on severity)
What is MAG3 or DTPA renal imaging good for showing?
Obstruction

e.g shows one kidney emptying slower than the other
____% of febrile children have UTI
~5
At what age are UTIs more common in boys?
6-12mo
Which organisms (5) cause UTI?
#1?
#2?
#1 E.Coli (80%)
#2 Klebsiella (10-15%)

Enterobacter
Proteus
Pseudomonas (if abnormal urinary tract)
Rx UTI (>12mo) (empiral ABx) (2 choices)
For how long if it's cystitis, and for pyelonephritis?
cephalexin
or
bactrim PO

3d cystitis (afebrile)
7d pyelonephritis (febrile)
Rx severe UTI (septic, vomiting) + for all <1mo (2)
ampicillin
+
gentamycin IV
What is the best specimen collection form for Dx of UTI?
clean catch

(in out catheter if really sick)
What would you see on UA for Dx of UTI?
Nitrite +
Leukocyte +

(if both then 96% sure, if just one is +ve then 70% sure)
What do you see on urine microscopy and culture for UTI?
WBC > 100x10^6/L
single organism > or = 10^8 (voided)
> or = 10^7 (catheter)
By definition, if a child with UTI is febrile, what do they have?
pyelonephritis
By definition, if a child with UTI is afebrile, what do they have?
cystitis
Preventative measures for UTI (6)
1. regular fluids/voiding
2. avoid/treat constipation
3. avoid caffeinated drinks
4. cranberry supplements
5. correct wiping
6. ?probiotics
True or false:
if VUR, circumcision can prevent UTI
true
If an infant <6mo has a typical UTI, what other Ix do you need to do? When?
Ultrasound within 6wks
Nephrotic or nephritic:

oedema and proteinuria
nephrotic
Nephrotic or nephritic:

haematuria
nephritic
Nephrotic or nephritic:

HTN
nephritic
Nephrotic or nephritic:

red cell casts
nephritic
Nephrotic or nephritic:

raised creatinine
nephritic
Most kids (80%) with nephrotic syndrome have which disease?
What age is it most common?
How is it treated?
Minimal change nephrotic syndrome
2-6 years

Prednisone PO 60mg/m2 for 4wks
then 40mg/m2 on alt days for 4wks
wean over 1-5mo
When would you do a renal Bx in nephrotic syndrome?
if after one month they haven't responded to steroids
Other than prednisone, what else is in the mgmt of nephrotic syndrome? (4)
1. fluid restrict til in remission
2. no added salt diet
3. pneumococcal vaccine
4. annual flu vaccine
What are 4 potential complications of nephrotic syndrome?
1. infection (urinary loss of IgG, T cell dysfunction, decreased C') - particularly encapsulated organisms (pneumococcus)
>> At risk of sponaneous bacterial peritonitis (S. pneumoniae most common)
2. thromboembolic (increased synthesis of clotting factors, loss of coag inhibitors into urine)
3. cardiovascular (since increased cholesterol)
4. drug S.Es
What organism usually involved in post infection GN?

What's the best test for proving an infection with this?
GAS

anti-DNase B titre
or
ASOT (Ab made against streptolysin O which is a toxin produced by GAS)
C3 is _________ in nephrotic
and __________ in nephritic
normal

low
DDx for acute nephritis (6)
1. post-infectious GN
2. IgA nephropathy
3. HSP
4. membranoproliferative GN
5. RPGN
6. SLE (ANCA +ve)
Rx options for nephritis (3)
nil
diuretics (Lasix)
antihypertensives
Mgmt of ARF
(6)
(depends on cause)
1. fluid correction
2. correction of electrolytes
3. BP mgmt
4. removal of toxins
5. drug dosage adjustment
5. dialysis
What are 4 causes of chronic renal failure
1. renal dysplasia
2. obstructive uropathy
3. GN (incl FSGS)
4. reflux nephropathy
CRF:
What happens to:
urea
increases
CRF:
What happens to:
Potassium
increases
CRF:
What happens to:
BP
increases
CRF:
What happens to:
Calcium
decreases
CRF:
What happens to:
PO4
increases
CRF:
What happens to:
Hb
decreases
HTN in kids = SBP or DBP >_____th centile
95
Broad causes of HTN in kids (6)
1. renal
- parenchymal eg. GN, polycystic kidneys, scarring
- obstruction
2. vascular
3. endocrine
- adrenal, thyroid, parathyroid, pituitary
4. metabolic syndrome
5. central
- e.g raised ICP, systemic nervous sys abNo.
6. iatrogenic
- drugs, IV fluids
What are the first Ix you get in a non-obese child or adolescent with HTN? (5)
1. EUC
2. CMP
3. UA
4. doppler U/S
5. ECHO
6 symptoms of anaemia
1. none
2. pallor
3. lethargy
4. failure to thrive
5. jaundice
6. red urine
Pale and jaundiced = _____________________

Pale and jaundiced and red urine = _________________
haemolysis

intravascular haemolysis
4 DDx for intravascular haemolysis
1. G6DP def
2. paroxysmal cold haemoglobinuria
3. malaria
4. ABO incompatibility
What do you think of in a well child, +/- recent Hx of viral infection, with sudden onset of petechiae and purpura?
ITP
How do you Rx acute ITP?
none normally necessary

resolves on own

may need IVIG or prednisone
What do you see on FBC in ITP?

Film?
decreased platelets

normal or large platelets, but <20 000/mm^3
How long would you give iron S' for for Fe def anaemia?
at least 3mo
What two things are a marker of bone marrow stress/infiltration?
1. raised MCV
2. HbF
Rx for very high WBCs

Why?
immediate IV fluids and platelets

high WCC can --> leukostasis --> intracranial bleed
What do you think of with anaemia, infection, bruising and gum hypertrophy?
AML
Waist:Height ratio
What's the aim?
<0.5
(ie keep your waist to less than half your height)

relatively independent of age (when >6)
How many Australian kids are overweight or obese?
23%

(1 in 4)
Are there any obesity drugs can be used in adolescence?
None are licenced
4 things in the pathology of arthritis
1. synovitis
2. bone erosion
3. pannus
4. cartilage degradation (joint space narrowing)
JIA classification criteria (3)
1. onset <16 y/o
2. duration > or = 6wks
3. exclusion of other causes of arthritis

(there is no diagnostic test)
What do you thin kof with fever, pain, tenderness, swelling, redness of a joint?

Ix?
Septic arthritis

joint aspirate and culture
blood culture
What do you think of wth fever and pinpoint bone tenderness?
Osteomyelitis
What is the major criteria for diagnosing Acute Rheumatic Fever?
"JONES" criteria
J: joints (arthritis)
O: looks like a heart (carditis)
N: nodules, subcutaneous
E: erythema marginatum
S: Sydenham's chorea

(OR "SPACE":
Subcut noducles, Pancarditis, Arthritis, Chorea, Erythema marginatum)
What are the minor criteria for Acute Rheumatic Fever? (4)
Fever
Arthralgia
Increased ESR, CRP
Prolonged PR interval
What do you need to Dx Acute Rheumatic Fever?
evidence of recent strep infection
+
2 x major
or
1 x major + 2 x minor
How long after an infection do you get post-infectious/reactive arthritis?
1-3weeks
What are the causes (3) or reactive arthritis?
GAS
enteric (eg salmonella)
viral: 'transient synovitis'
3 causes for hip pain arranged by age
2-6 years old: transient synovitis
4-10: Legg-Perthe's
10-14: Slipped Epiphysis
What is Legg-Perthe's disease?
What will you see on Xray?
idiopathic avascular necrosis of the femoral head

flattened femoral head
Who gets slipped capital femoral epiphysis?
older obese boys
Where does hip pain refer to?
knee
Most common organism in septic arthritis and osteomyelitis?
S. aureus
If there is joint pain and refusal to weight bear, think of.... (4)
1. septic arthritis/osteomyelitis
2. malignancy
3. reactive arthritis
4. pain syndromes
If there is joint pain at night think of...(3)
1. malignancy
2. osteoid osteoma
3. growing pains
What comes under the umbrella of Juvenile Idiopathic Arthritis? (6)
1. Systemic arthritis (Still's)
2. Oligoarthritis (< or = 4 joints)
3. Polyarthritis (> or = 5 joints)
4. Enthesitis related
5. Psoriatic arthritis
6. Unclassified
What happens to the fever in systemic JIA?

What coincides with the fever?
"Quotidien" fever - spikes and returns to below baseline

Salmon coloured rash
Oligoarticular JIA:
age:
which joints? Which is most common?
<5years

large joints
knee most common
Oligoarticular JIA:

ANA: +ve or -ve?
RF: +ve or -ve?
ANA +ve in 80%

RF -ve
What do you worry about in oligo JIA? (3)
1. knee flexion contracture
2. quadriceps atrophy
3. leg length discrepancy
What else do you have to screen for in JIA, particuarly in oligo? (it's associated with ANA +ve)
Uveitis
(it's asymptomatic)
RF -ve Polyarticular JIA:
which joints?

ANA +ve or -ve?
small and large joints, neck, TMJs

ANA +ve in 50%
What do you worry about in RF -ve poly JIA? (3)
1. cervical spine arthritis
2. TMJ arthritis ('chipmunk')
3. growth distrubance
RF +ve JIA:
which joints?
What else do they get?
small and large joints

rheumatoid nodules over pressure points (30%)
Seronegative spondylarthropathies:
arthritis where?
ANA and RF +ve or -ve?
peripheral and axial arthritis

ANA and RF -ve
What HLA type in seronegative spondyloarthropathies?
HLA-B27
Enthesitis fits into which category?
seronegative spondyloarthropathies
Is the probability of having active disease into adulthood with JIA high or low?
high
What is HSP short for?

What sized vessels does it involve?

Which parts of the body does it commonly affect? (4)
Henoch-Schonlein Purpura
= small vessel vasculitis (venules, capillaries)

skin (scalp, scrotum), joints, GIT, kidneys
What are the GI complications assoc with HSP?
bleeding
intussusception
Diagnostic criteria for HSP (4)
Have to have at least ____
Have to have at least two of:

1. palpable purpura
2. age <20 at onset
3. bowel angina (abnormal pain after meals or bowel ischaemia usually w/ bloody diarrhoea)
4. granulocytes in walls of arterioles or venules on Bx
DDx for HSP (4)
1. acute abdomen
2. acute scrotum
3. meningococcal disease
4. other vasculitis e.g PAN (Polyarteritis nodosa)
Rx: HSP
symptomatic (NSAIDs)

severe abdo pain may need steroids

Urine/BP needs F/U
Dx: SLE (hint: mneumonic)
"MD Soap 'n Hair"
Malar rash
Discoid rash
Serositis (pleuritis, pericarditis)
Oral ulcers
Arthritis
Photosensitivity
Neuro disorders (Sz, psychosis)
Haematologic disorder
ANA positive
Immunologic disorder (Lupus erythematosus prep test, anti-dsDNA, anti-Smith)
Renal disorders
What is Juvenile dermatomyositis?
What are the symptoms?
an inflammatory myopathy of unknown cause that affects muscle, skin and blood vessels.
Photosensitive voilet coloured or dusky red rash around extensor surfaces and on face plus proximal progressive muscle weakness
What do you see in nail fold capillaries when magnified
in Juvenile dermatomyositis?

What's a complication of JD?
tortuous capillaries

deposition of calcium in the skin (calcinosis)
What is scleroderma?

What are the symptoms? (3)
connective tissue disorder causing bands of thickened skin

1. thickened skin
2. Raynaud's
3. stiffness and pain in joints
4. GIT: indigestion, diarrhoea, constipation
What is the most common cause of hip pain in a school aged child?

When should you do Ix?
Transient synovitis (reactive arthritis from viral URTI)

Ix only if hip pain persists >10d
What is the key sign to Perthe's disease?
asymmetry with ROM of hips
What is the Rx for Perthe's disease?
Rx: bisphosphonates to maintain femoral head shape so it doesn’t collapse- eventually revascularises
What is slipped capital femoral epiphysis?
proximal displacement of femoral neck
Which side does growing pain occur on?
always bilateral
Most common symptoms of oligoarthritis (2)
morning stiffness
limping
What causes Still's disease?
it's an autoinflammatory disease (no autoantibodies; it's the innate immune system)
Most common sites of Enthesitis (4)
1. tibial tuberosity (achille’s tendon)
2. plantar fascia insertions on base of foot
3. insertion of glut max onto ischial tuberosities
4. anterior superior iliac spine.
What is the HEADS interview?
"HEEADSSSS"
Home
Education and Employment
Exercise, Eating
Activities - include peers and risky behaviour
Drugs - including EtOH, cigarettes
Sexuality
Suicidality + mood
Safety - from injury at school, work, home, with friends
Sleep
What ages does 'adolescence' encompass?
10-19
What is the #1 reason for female young people (age 12-24) for hospitalisation?
For male?

Followed by what?
pregnancy and childbirth

injury and poisoning

#3: digestive system problems
#4: mental and behavioural disorders

Chronic illness also is a major cause of adolescent morbidity
What are the developmental tasks of adolescence? (7)
1. est realistic body image
2. est self identity
3. achieve independence
4. integrate into peer group
5. become comfortable with own sexuality
6. acquire skills for future vocation
7. develop value system

--> these tasks may be affected by illness
How much sleep does the average 16 year old need?

Required sleep _______ during adolescence
8.5h

declines
Which phase of sleep is reduced in adolescence?
slow wave sleep
= less efficient/restorative sleep
Adolescents sleep later and wake later. They have less efficient sleep. They do not get enough sleep.

What are the consequences of this? (6)
1. increase risk of injury
2. poor s
3 things used to Dx sleep disorder
1. sleep diary -- helps identify trends in problems of sleep/wake cycle
2. actigraphy -- worn on wrist, records motion - used with diary
3. Polysomnogram -- good when OSA suspected or symptoms can't be explained by other 2. Measures brain waves, eye movement, HR etc...
What are 4 common sleep disorders in adolescence?

Which is most common?
1. poor sleep hygeine (most common)
2. delayed sleep phase disorder
3. insomnia
4. sleep disordered breathing
What are 6 things that contribute to poor sleep hygiene?
1.
What is Delayed Sleep Phase Disorder?

Mgmt?
A circadian rhythm disturbance
-- unusually long circadian rhythm
- sleep onset and wake times intractably later than desired
- extreme difficulty waking in the morning

Mgmt: bring sleep time 1/2h earlier each week, improve sleep hygiene, walk 20min in sun w/o sunglasses each morning, no naps, exercise, melatonin only in refractory cases
How common is insomnia in adolescence?
point prev = 4-5%
3 causes of insomnia in adolescence?
1. primary (use HEADS to explore)
2. part of psych disorder
3. assoc with drugs/meds
What causes the most burden of disease and disability in 10-14year olds? (4)

In 15-19 year olds? (3)
1. mental health - Anx + Dep
2. chronic illness and disability
3. reducing risks for future ill health
What are the two systems GPs most commonly treat in young people?
1. respiratory
2. skin

(social problems is last)
What is the prevalence of mental health problems in young people?
one in four
What supports resilience in adolescents? (4)
1. connections to partents, family, school,
Moles in kids change.
When are they a concern?
If they're changing and the kid is not growing
Moles start off as ___________ ______________ which are flat and dark.
Over time progresses down into dermis to become _________________ _______________
Over time becomes less pigmented and are called _________________ _______________
Junctional naevi

Compound naevi

Dermal naevi
What is it called when there is a very big naevus on a baby.
Worry if it crosses midline because ________________
____% will get melanoma before age 5
Bathing trunk naevus

there might be something in the spine

50%
What surfaces do you usually get eczema on?
What is the mutation in eczema?
Rx of eczema? Why?
ventral
'Lagran'
moisturise 3xday to keep skin barrier in tact so allergens don't get in
May need wet dressings to help absorb if severe eczema
Rx for eczema flare
as well as mois
Dx:
yellow and crusty
usually in first 3mo of life
not itchy

Rx?
Seborrheric dermatitis

Rx: moisturise and weak steroid
How do you tell the difference between allergic contact dermatitis and periorbital cellulitis?
contact dermatitis: well child
periorbital cellulitis: unwell child
A singlular annular lesion = _______________ til proven otherwise


Next step?
TINEA


do skin scraping for fungal microscopy
What is the name for ring worm?

From exposure to what?
Tinea corporis

cats, guinea pigs, rabbits
Is tinea asymmetrical/symmetrical?
Unilater/bilateral?

And psoriasis?
Tinea: asymmetrical and unilateral

Psoriasis: symmetrical and bilateral
What is endothrix?

Ectothrix?

What condition do you get it in?
endothrix: broken off hairs at the surface of scalp= black dot tinea. spores inside hair shaft

ectothrix:hairs broken off above surface of scalp, so spores are outside hair shaft

Tinea capitis
What do you think of when you see alopecia and black dots on scalp?
It's itchy.
tinea capitis
What is a Kerion?
What do you get it in?
Rx?
inflammatory response - has elevated, boggy, granulomatous mass
Tinea capitis
Rx: debridement
What do you think of with GROUPED lesions, well demarcated, SCALLOPED edge, painful. Blister.
Rx?
HSV

Rx: IV fluids, acyclovir
What's it called when HSV targets areas of eczema?
What do the lesions look like?
Eczema herpeticum

('punched out' lesions with scalloped edge and grouped lesions)
shingles

If tip of nose is affected => __________ branch of facial nerve is affected => look for ____________=> send to ___________________

Rx: ?
opthalmic
uveitis
opthalmologist

IV acyclovir
What is the causative organism for molluscum contagiosum?

how do the lesions appear?
How is it spread?
Rx?
Pox virus
skin coloured, cone shaped, with central umbilication (depression in middle)
spread from direct contact or in warm water
Rx: Imiquimod or prick them (both simulate inflamm response)
What do you think of with golden eruptions that look like cornflakes (honey coloured, crusted plaque)
Non-bullous staph impetigo
What do you think of with pus filled lesion?
Bullous impegito
Rx for impetigo?
Fluclox PO
What do you think of with pustules on soles of feet and on palms?
Intense pruritis.
= SCABIES til proven otherwise
Rx: Scabies
topical PERMETHRIN 5%

treat whole family at same time on 2 consecutive nights for 2 consecutive weeks
Wash all clothes and linen in hot water
What do you think of:
in a tanned patient, get pale lesions, in a pale patient, get orangey pigmentation?

What causes it?
What exacerbates it?
Pityriasis versicolor

Malassezia furfur (yeast)

humid, hot weather
Rx: antifungals
What causes scabies?
Sarcoptes scabiei
What do you get if you have multiple pinna haematomas that don't heal properly?
Cauliflower ear
3 organisms assoc with otitis externa
1. P. aeruginosa (in water)
2. S. aureus (on skin)
3. Fungal
Why do you get otitis externa?
thin skin in ear canal --> while wet, traumatise skin --> bacteria gets in

happens a lot in summer when swim a lot
Otitis externa: what do you see?
swollen, macerated ear canal
How do you differentiate otitis externa from mastoiditis?
OE: hurts when pull on ear; ear canal is red and swollen and can't see ear drum

mastoiditis: doesn't hurt when pull on ear; ear drum visible and is red and bulging
Rx: otitis externa
may need cleaning with microscope, wicks or packing
Topical drops/creams/ointments
e.g Sofradex = combo of steroids, ABx and antifungals
Why is otitis media common in childhood? (2)
Poor aeration due to less palatal muscle mechanics and strength - this leads to -ve pressure which draws fulid in and holds it there with URTIs
Poor drainage from eustachian tube due to shorter tube and horizontal axis - this keeps the fluid there

(interruption of normal eustachian tube function [ventilation] by obstruction -> inflamm response -> middle ear effusion [secretions from mucous secreting cells that line the middle ear]-> infection (most with URTI)
OM can be caused by viruses or bacteria.
What are the 3 most common bacteria?
1. Streptococcus pneumoniae
2. Haemophilus influenzae
3. Moraxella catarrhalis
Rx for OM
No systemic illness:
6mo - 2yrs - analgaesia only and r/v in 24h - if persists, give ABx
2yrs+ - as above but r/v after 48h
Systemic illess or <6mo:
ABx - AMOXICILLIN and analgaesia
Otoscopic signs of OM (5)
1. bulging/fullness of TM
2. intense erythema
3. some degree of opacity (indicates effusion)
4. loss of light reflex
5. pneumatic otoscope: blow air in and see little to no movement of TM
What do grommets do?
When would you put them in?
= pressure equalising tube
lets air into middle ear space

OM with effusion (if doesn't resolve after 3mo)
What is chronic suppurative OM?

Rx?
perforated TM with persistent drainage from middle ear

Rx: keep dry (dry mop with tissue spears = aural toilet)
topical drops (Ciprofloxacin)
What is it called when there is squamous epithelium proliferation in the middle ear?
Rx?
Cholesteatoma
(this is the other type of chronic suppurative OM [cf tubotympanic])

Surgical Rx
4 complications of OM
1. CNVII damage (facial nerve palsy) from damage to middle ear structures
2. localised abscess (mastoiditis)
3. suppurative labrynthitis (deafness, dizziness) from damage to inner ear
4. intracranial - extradural abscess, meningitis, subdural abscess, i/c abscess
What do ears look like in mastoiditis?
pushed out
Congenital Sensorineural hearing loss
Rx?

Due to damage of cochlear
hearing aids

Cochlear implant if severe
What area usually bleeds in the nose to cause epistaxis?
Rx?
anterior: "LITTLE'S AREA"
= thinned, dried out mucosa
Hesselbach's vessels rupture easily
Cover with ointment to thicken mucosa
2 reasons the nose can be obstructed
1. allergy (increases turbinates size)
2. enlarged adenoids
Indications for tonsillectomy and adenoidectomy (2)
1. tonsillitis:
6x past year
4-5x past 2 yr
3x past 3 yr
2. OSA
Complications of tonsillectomy/adenoidectomy (3)
1. GA
2. haemorrhage
3. palatal incompetence (causes nasal air escape which makes talking difficult)
To avoid inhaling a FB, it is advised to avoid small foods like nuts until the age of ___
7
What do you see on CXR with FB inhalation?
air trapping
Rx: inhaled FB
bronchoscopy and removal
Three main components causing the pathophysiology of asthma
1. airway inflammation and oedema
2. bronchospasm
3. mucous production
Prevalence of kids in Australia with asthma
10-15%
Are all asthmatics atopic?
no
(usually is though; 80%)
Definition of asthma
repeated episodes of REVERSIBLE bronchoconstriction
What symptom MUST be present in order to Dx asthma?
wheeze

(but not all that wheezes is asthma - transient wheeze that usually comes on with viral infection and remits without intervention)
What are the 6 steps in the Six Step Asthma Mgmt Plan?
1. assess severity
2. achieve best lung fn
3. maintain best lung fn - avoid triggers
4. maintain best lung fn - optimise meds
5. develop asthma action plan
6. educate and r/v
What severity of acute asthma is this -
Altered consciousness: No
SaO2: 90-94%
Talks in: phrases
PR: 100-200
Central cyanosis: Absent
Wheeze: mod-loud
Moderate
What severity of acute asthma is this:
Altered consciousness: agitated/drowsy/confused
SaO2: <90%
Talks in: words or unable to speak
PR: >200
Central cyanosis: likely
Wheeze: often quiet
Severe/lifethreatening
What severity of acute asthma is this:
Altered consciousness: No
SaO2: >94%
Talks in: sentences
PR: <100
Central cyanosis: absent
Wheeze: variable
Mild
What is asmol?
salbutamol
What is the dose of salbutamol in acute asthma mgmt?
<20kg:
6 puffs (600mcg) via spacer or 2.5mg via nebuliser

>20kg:
12 puffs (1200mcg) via spacer or 5mg via nebuliser
What is atrovent?
ipratropium bromide
(anticholinergic)
Dose of ipratropium bromide in acute asthma mgmt?
<20kg:
4 puffs then q6h or 250mcg q20min x 3 via neb then q6h

>20kg:
8 puffs then q6h or 500mcg q20min x 3 via neb then q6h
In acute asthma mgmt, after giving salbutamol and ipratropium bromide, if this fails, what do you do next? (6 options in order)
1. IV salbutamol infusion
2. IV magnesium sulfate bolus
3. Aminophylline
4. Anaesthetic agents
5. CPAP
6. intubation and ventilation
Who should get predisone in acute asthma?
those who had severe wheeze who required hospital admission
What is the dose for oral prednisone in acute asthma mgmt?
Initial dose: 2mg/kg PO (max 60mg)
Followed by: 1mg/kg PO daily for 3days
Criteria for d/c after acute asthma?
Salbutamol q3h

(after having stretch the salbutamol administration)
What should you give parents when discharging a child for acute asthma? (5)
1. 'reducing medication' plan
2. asthma action plan
3. d/c meds
4. F/U arrangements
5. The Children's Asthma Resource Pack for parents and carers
How do you diagnose asthma?
spirometry (if mental age >4-6 y/o)
CXR (but this is not routinely done after first presentation)
What Rx is needed for infrequent intermittent asthma?
(exacerbations < every 4-6wks, nil night time symptoms, FEV1 >80%)
just salbutamol
What Rx is needed for frequent intermittent asthma?
(exacerbations >2per mo, nil night time symptoms, FEV1 >80%)
salbutamol
+
preventer
Can you use long acting beta2 agonists on their own in paeds?
NO
must use with ICS
Preventers are prescribed twice per day, except for:
2 which are prescribed once per day.
Which two?
and
1 which is prescribed three times per day.
Which one?
Singulair (Montelukast)
Alvesco (Ciclesonide)

Intal Forte (Sodium Cromoglycate)
What is flixotide?
fluticasone
What is Pulmicort?
Budesonide
What is Qvar?
Beclomethasone
What is Seretide?
Fluticasone and Salmeterol
What is Symbicort?
Budesonide and Eformeterol
What is Intal Forte?
Sodium Cromoglycate
= preventer
Important to note that with all preventer therapy there is the potential for _________ __________ when the child's asthma is clinically stable
back titration
What 5 things indicate good asthma control?
1. minimal Sx during day and night
2. minimal need fo reliever medication
3. no exacerbations
4. no limitiation to activity
5. normal lung fn: FEV1 and/or PEFR >80% pred
Inhalation delivery devices: which would you choose for-
age <4
small spacer with mask
Inhalation delivery devices: which would you choose for-
4-6yrs old
large or small spacer
no mask necessary
Inhalation delivery devices: which would you choose for-
age >6
(3 choices)
Turbuhaler
Accuhaler
Autohaler
Inhalation delivery devices: which would you choose for-
age >8
Puffer (no spacer - but spacer is preferred, esp for ICS)
Asthma action plan:

When to seek medical advice (2)
- reliever meds every 3-4h

- given oral steroids with no/little improvement
Max dose for reliever meds
<20kg: 6 puffs
>20kg: 12 puffs
How long after having salbutamol do you wait before doing spirometry again?
10mins
What is a positive bronchodilator response on spirometry?
FEV1 > or = 12% and > or = 220mL
FEV1 for mild, mod and severe obstructive disease

(the same parameters apply for FVC when assessing restrictive disease)
mild: >60%
mod: 40-60%
severe: <40%
Normal sleep patterns for 0-3mo
95% sleep during the day every day
take 0.5-1h to settle
may wake up to 6 times/night, most commonly twice
normal sleeping patterns for 1 year old
89% have daytime nap for 1-2h
most settle b/t 6 and 8pm
80% settle quickly
60% have night time waking requiring settling by parent, 10% wake more than 3xnight
Normal sleeping patterns for 2 year old
half have daytime naps
stay up later - 1/3 sleep after 8pm
1/4 take >30min to settle
1/2 wake once during night requiring parent's attention
Is regurgitation after a feed normal?
yes, 2/3 report it at 4mo
most outgrow by 12mo
When does reflex become pathological? (4)
- poor growth
- blood stained (oesophagitis)
- breathing difficulties, chest infection
- crying, irritability
Rx of reflux? (4)
1. none required for most kids
2. thickened feeds
3. PPI
4. fundoplication if severe
What do you think of with loose frothy stools? (3)
1. Lactose intolerance (due to relative lactose malabsorption)
2. oversupply (advise to feed from 1 breast only for a few feeds)
3. xS CHO-rich fore-milk and not enough fat-rich hind milk: hind milk slows absorption and allows time for digestion so advise to allow baby to fully feed from one breast
What is the most common cause of lactase def in infancy?
Secondary Lactase Deficiency
-- results from small bowel injury (e.g gastro) -- knocks off brush border

Primary Lactase Def is the most common cause of lactose intol overall but uncommon <2years of age. This is a relative or absolute absence of lactase.
What sort of lactose intolerance can premature babies get?
'Developmental lactase deficiency'
seen in <34wks gestation
Ix for lactose intol? (6)
1. 2wk trial on lactose free diet and challenge
2. hydrogn breath test in subtle cases
3. stool tested for pH (low) and faecal reducing substances
4. intestinal Bx (measure lactase activity)
5. stool specimen for Giardia or cryptosporidium
6. check Ab for coeliac disease (TTG, total IgA)
Should infants who are being breastfed and who are lactose intolerant continue to breastfeed?
yes
How common is congenital lactose intolerance?
very very rare
What weight gain should you expect to see in an infant?
100-200g/week
A toddler should be drinking no more than _____mL of cow's milk/day
600
What is the most important sign of adequate nutrition?
growth monitoring
When introducing new foods, it may take up to ____ tried before the new food is accepted
10
Define constipation
delay or difficulty passing stool present for >2wks
How often do normal babies poo?
First wk of life: 4xday
By age 2: 1xday
Do breastfed or formula fed babies poo more?
formula

it's normal for some breast fed babies to go for several days without doing a poo
Most common cause of constipation in kids?

Rx:?
Functional constipation
e.g not wanting to go, forgetting

Rx: <1yr: prune juice, lactulose, glycerine suppository
>1yr: mineral oil, lactulose, glycerine suppository
Do you get a fever when teething?
only a mildly raised fever
What is Positional plagiocephaly?

What side is it normally on?
What exacerbates it?
posterior skull flattening, uni or bilaterally
Ipsilateral frontal bulge
forward displacement of ipsilateral ear

RHS
Sleeping on back
DDx of positional plagiocephaly? (2)
1. craniosynostosis (has palpable ridging over prematurely fused suture)
2. congenital muscular torticollis
Is head circumference normal or abnormal in positional plagiocephaly?
normal
What is the difference b/t Primary and Secondary nocturnal enuresis?

What causes it?
Primary: never fry for more than few months
Secondary: was dry for >6mo and then wets again

cause: ?
Nocturnal enuresis: when should you seek professional help?
if still wetting >age 6
When is toilet training usually done?
How long does it take?
usually successful from 2.5years

~2weeks
In the feotal circulation, what vessel connects the umbilical vein to the inferior vena cava, and in doing so shunts 1/3 of the blood past the liver?
ductus venosus
What is the valve called in the foetal circulation that directs blood straight towards the foramen ovale?
euestacian valve
What happens to foetal circulation after a baby is born?
breath --> decrease in pulmonary vascular resistance --> increase in systemic vascaulr resistance --> closure of 3 foetal systemic-pulmonary shunts: DA, foramen oval, ductus venosus
When does the PDA close after birth?
- functionally
- permanently
w/i 10-15h

w/i 2-3wks
When does RV dominance diminish?
slowly throughout childhood years
LV is dominant as an adult
(the point of this is that there is a difference in normal ECG in kids with RV leads at precordium looking larger)
At what age do you have max HR?
~3mo

HR initially increases after birth and then decreases after 3mo
Kawasaki disease - AKA ___________________________
mucocutaneous LN syndrome
What cardio effects do you worry about with Kawasaki disease?
coronary artery aneurysms
What poses the highest risk for contracting endocarditis?
cyanotic heart diseae with posthetic shunt
Who gets endocarditis prophylaxis? (5)
1. prosthetic cardiac valves
2. previous IE
3. post cardiac Tx with cardiac valvulopathy
4. RHD with valvular pathology
5. selected CHD
Incidence of congenital heart disease?
1/100
4 chromosomal abnormalities assoc with congenital heart disease
1. Down Syndrome
2. T13
3. T18
4. Turners
Most common congenital heart disease?
VSD
(1/3)
5 Genetic syndromes assoc with congenital heart disease
1. Velo-cardio-facial (22q11 del)
2. Marfan syndrome (Fibrillin gene)
3. Williams Syndrome (elastin gene)
4. CHARGE association
5. VACTERL association
2 maternal diseases/infections that can --> congenital heart disease
1. SLE
2. rubella
What are the 3 commonest reasons for a baby to collapse in first week of life?
1. - infection --> GBS
2. - obstructive left heart lesions (e.g coarctation)
3. - inborn errors of metabolism e.g hypoglycaemia
How many children with Down Syndrome have AVSD?
50%
What do you think of with absent femoral pulse?
coarctation

might still be there though on day one if ductus still open
What sign is come common than peripheral oedema in kids with RH failure
hepatomegaly
duct dependent pulmonary circulation - if closes, will --> ______________

duct dependent systemic circulation - if closes --> _______________________
cyanosis


shock and collapse
Four causes for clubbing
1. cyanotic heart disease
2. IE
3. IBD
4. congenital familial disease
Grades of murmurs
1: scarcely audible, no thrill
2: soft, no thrill
3: loud, no thrill
4: loud, faint thrill
5: very loud, easily felt thrill
6: audible without stethoscope
Physiologically, can you hear a split S2 during....?
inspiration
- increased pulmonary venous return to right side of heart delays closure of pulmonary valve
What are the features of an innocent murmur? (5)

NB there are NO symptoms
1. systolic ejection
2. Grade 1-3
3. musical, vibratory
4. change with body position
5. augmented with illness
4 innocent murmurs
1. Still's
2. pulmonary flow
3. venous hum (blood coming back into SVC)
4. carotid bruits (be sure there's no aortic pathology)
CXR: what do you see in VSD? (2)
1. cardiomegaly
2. increased pulmonary vascular markings (from pulmonary venous congestion)
CXR: TOF (3)
1. absent artery component
2. boot shaped heart
3. oligaemic lungs (decreased pulmonary vascular markings)
What do you see on ECG in normal newborn vs normal adult?
right axis deviation

RV dominance -- big R wave in V1, small R wave in V6

adult: LV dominance -- big S wave in V1, big R wave in V6
4 left to right shunt diseases
what category do they fall into?
acyanotic

1. ASD
2. VSD
3. AVSD
4. PDA
What are the cyanotic heart diseases (5Ts!)
1. TOF
2. Transposition of great aa
3. Truncus arteriosus
4. Tricuspid atresia
5. Total anomalous pulmonary venous connection
What do you worry about long term with ASD?
long term worry about pulmonary HTN
- LA pressure goes up because more blood coming back as more going into lungs
CXR: ASD
RV and RA enlargement
Murmur in ASD?
Systolic ejection murmur - from incr pulmonary flow
Wide fixed splitting of S2 - R heart getting a bit more extra blood so R heart takes a little bi tlonger to empty and so pulmonary valve takes a bit longer to snap shut
CXR: VSD
left heart enlargement
TOF: made up of...
"VORP"

VSD
Overriding aorta
RV hypertrophy
Pulmonary and infundibular stenosis (RV outflow obstruction)
Transposition of great aa - which two things do you need open?
1. PDA
2. foramen ovale (can do artificially)
What do you give to keep PDA open?
PGE1
What happens in hypoplastic left heart syndrome when PDA closes?

What is the Rx?
baby collapses

operation to get RV to maintain both pulmonary and systemic circulation (Norwood stage 3 procedure)
What is a tet spell?
Rx?
acute onset of hypoxia --> incr cyanosis --> gasping and syncope

Rx: place in lateral knee-chest position (increases systemic vascular resistance and therefore decreases R--> L shunt, give O2
What genetic condition is associaed with ventricular tachycardias?
long QT syndrome
What do you think of with:
epiphora and discharge of eye since birth
mucocoele of lacrimal sac
Congenital nasolacrimal duct obstruction
3 causes of epiphora
(xs tear production)
1. Congenital nasolacrimal duct obstruction
2. Congential glaucoma
3. Corneal Irritation (FB or lashes)
Rx: Congenital nasolacrimal duct obstruction
bathe with saline and massage over lacrimal sac
probe and irrigate if not resolved by age 1
4 causes of neonatal conjunctivitis
Which comes when?
1. Gonococcal (typically presents d1)
2. viral
3. other bacteria (d4-6) (staph, strep)
3. Chlamydia (presents around d10 post NVD)
How do you Rx neonatal conjunctivitis? (2)
1. topical ABx for staph/strep
2. systemic ABx for gon/chlam


and remember to treat the parents!
What do you think of with bilateral sticky eyes and eyes injected with mucopurulent d/c?
Rx?
Bacterial conjunctivitis

(viral would have clear d/c)
Rx: topical ABx
What do you think of with vesicles involving upper and lower eyelids + watery eye
Herpes Simplex Blepharo-Conjunctivitis

Rx: topical acyclovir
What do you think of with watery and irritable eye assoc with photophobia and injected eye?

Rx?
Fluorescein

Blue-filtered light on slit lamp
If you have an irritable eye, put fluorescein in and see an ulcer, what do you think of?

Rx?
Herpes Simplex Keratitis
Rx: acyclovir
How do you test visual acuity in a preverbal child (age 0-2)?
response to small toys - see if they fix and follow
response to 100s and 1000s
What do you use as a screening program for visual acuity in pre-school children?
Sheridan Gardiner test:
matching test - points to symbol on their card that matches what you're pointing to on the screen
From what age can you use the Snellen Chart for visual acuity?
5
What do you think of with abnormal head posture?
strabismus
What is the Hirschberg test and what's it used for?
Light reflex should be symmetrical in both eyes

Used for Dx of strabismus
What do you use the cover-uncover test for in strabismus?
esotropia (eye turning out) - shows up at near - eyes look straight when looking into distance, and deviated when looking at something close up

exotropia - shows up at distance - eyes deviate when looking in distance but straight when looking at an object closely
What is the term for 'lazy eye'?
amblyopia
What is the most common cause for amblyopia?


Rx of amblyopia?
refractive errors in one eye - use good eye and ignore eye with refractive error

(can also get from strabismus - ignore one eye to avoid double vision)

Rx: patch
What do you think of with sudden onset strabismus assoc with diplopia?
intracranial pathology
DDx: leucokoria (white reflex) (2)
1. retinoblastoma
2. cataract
What do you think of with leukocoria, strabismus, glaucoma and uveitis?
retinoblastoma
3 causes for congenital cataracts?
1. rubella
2. other TORCH infections
3. galactosaemia
What do you think of with retinal haemorrhage?
NAI
What do you think of with 'spontaneous ecchymosis' around eyes?
NAI
What is the only intervention proven for the Rx of reading difficulties?
remedial intervention
Which sort of rash:
circumscribed, elevated lesions, i.e bumps
Papules
Which sort of rash:
flat and impalpable
macules
Which sort of rash:
elevated lesions containing a purulent exudate
pustules
Which sort of rash:
circumscribed, elevated, fluid-filled and normally <0.5cm in diameter
Vesicles
Which sort of rash:
may be flat or raised
cannot be blanched
small, <0.5cm
caused by minor haemorrhage
petechiae
Which sort of rash:
may be flat or raised
cannot be blanched
>0.5cm
caused by minor haemorrhage
purpura
Petechiae: think of which 2 things
1. meningococcal
2. low platelets
What sort of rash is hand, foot and mouth?
vesicular
When does VZV stop being infectious?
when lesions crust (from 48h prior to onset of rash ti they crust)
Where are chicken pox vesicles predominantly located? (cf small pox)
trunk

(but they are everywhere)
When would you give VZIG infant?>
if mother has it 48h before - 5d after delivery -- may cause severe neonatal disease
DDx for vesicular rashes (7)
1. VZV
2. HSV
3. hand foot and mouth
4. impetigo
5. molluscum contagiosum
6. dermatitis herpetiformis
6. Stevens-Johnson syndrome
Who gets Rx for varicella? (3)
1. neonate
2. immunocompromised
3. complicated VZV
How does neonatal HSV infection present (4)
1. skin, eye, mouth lesions
2. encephalitis
3. disseminated infection (looks like septicaemia)
4. pneumonitis
What most commonly causes hand foot and mouth disease?
coxsackie virus 16
(enterovirus)
How do you tell the difference between HSV infection and enterovirus (Coxsackie) in the mouth?
HSV: involves gingiva, more anterior

Cox: gingiva not involved, more posterior (back part of mouth + soft palate)
DDx for maculopapular rashes (7)
1. measles
2. rubella
3. scarlet fever
4.
What do you get on the inside of cheeks in measles?
Koplik spots
(greyish white)
Dx: measles
PCR
What sort of rash in measles?
maculopapular
What's the difference between rubella and measles rash?

What else do you get in rubella?
rash in rubella develops more quickly and disappears earlier than in measles

the rash is less florid

LN enlargement: posterior, cervical
What organism causes scarlet fever?

Rx:?
GAS

Rx: Penicillin
What do you think of with strawberry tongue?
scarlet fever
What colour is the rash in scarlet fever?

What is a distinctive feature of scarlet fever?
dark red

circumoral pallor - rash spares area around mouth
Kawasaki disease criteria (6)
Fever for at least 5d plus 4/5:
- bilateral conjunctival injection
- rash
- oral changes -> red mouth/pharynx/tongue and cracked lips
- swelling of hands + feet then desquamation
- cervical LN enlargement (at least one >1.5cm)
What sort of rash seen in Kawasaki disease?
discrete red maculopapules
DDx for Kawasaki (2)
1. scarlet fever
2. staph disease
What are the other 2 names for Slapped Cheek?
What causes it?
Erythema infectiosum
Fifth disease

Parvovirus B19
Rash in Fifth disease
starts as red cheeks
then
lacy, maculopapular rash 1-2wks later
mainly arms and legs
Other than rash and fever, what else is associated with Fifth diseae?
joint aches
What causes Roseola infantum?

Where do you mainly get the rash?
What sort of rash?
HHV-6

maculopapular
trunk
What's the diff b/t the rash of roseola and measles?
measles: lesions become confluent
roseola: lesions are discrete
What do you think of with high fever 3-4d then disappears and get maculopapular rash?
roseola infantum
2 presentations of meningococcal disease
1. meningitis
2. septicaemia
Type of rash in meningococcal disease?
early on: maculopapular

then petechial/purpuric
What is the single best test for rapidly Dx bacterial meningitis?
CSF gram stain
Meningococcus> Gram ______ ______
negative diplococcus
Common causes of bacterial meningitis in neonates? (3)
1. GBS
2. E. Coli
3. Listeria monocytogenes
Common causes of bacterial meningitis in > or = 3mo - 16 years? (3)
1. N. meningitidis
2. Hib

3. Strep pneumoniae (any age)
Empiric ABx for suspected bacterial meningitis: neonate
Ampicillin
+
Cefotaxime
Empiric ABx for suspected bacterial meningitis: > 3mo
Cefotaxime
or
Ceftriaxone

Add vancomycin if suspect strep pneumoniae
On CSF for bacterial meningitis, what do you expect to see for:
PMNs
Lymphocytes
Protein
Glucose
PMNs: increased: 100- 100 000
Lymphocytes: <100
Protein: increased >1
Glucose: decreased
On CSF for viral meningitis, what do you expect to see for:
PMNs
Lymphocytes
Protein
Glucose
PMNs: <100
Lymphocytes: increased: 10-10 000
Protein: 0.4-1
Glucose: normal
What is H1N1?
Swine flu
What is H5N1?
Avian flu
What is common in TB in aged <4 years old?
CNS dissemination
If neonate presents with any of:
fever
lethargy
anorexia
apnoea
--what should you suspect?
infection
Any febrile newborn up til 3mo should have cultures of ____________, ______________ and ______________ and then should be started on empirical ABx
blood
CSF
urine
What is the commonest cause of congenital infection in Australia?

____% are asymptomatic. What do you worry about long term with this infection?
CMV

90
hearing loss
mental retardation
Triad of systems in congenital rubella
1. eye - cataracts
2. cardiac
3. CNS - deafness, microcephalic
Most common cause of viral gastro?
rotavirus
Dx on Hx of gastroenteritis (4)
1. increased watery stools
2. vomiting
3. fever
4. infectious contacts
When looking at a fatty stool, what two things do you look for and what do they mean?
1. Fat globules
- lipase def (e.g CF)
- liver disease

2. fatty acid crystals
- mucosal disease --> do small bowel Bx for histology
What chromosome is affected in CF?
what is the most common mutation?
7
delta F508
What are the signs of CF? (5)
1. meconium ileus -- bilious vomiting
2. chronic diarrhoea (from pancreatic insuff)
3. FTT
4. fat sol Vit def (ADEK)
5. resp illness
What 2 causes of chronic liver disease causes steatorrhoea?
1. extrahepatic biliary atresia
2. cirrhosis

- decreased intraluminal bile salts - impaired fat digestion
Who gets regular screening for Coeliac disease? (6)
1. Fe def anaemia
2. IgA def
3. Down Syndrome
4. Turner syndrome
5. Williams Syndrome
6. 1st degree relatives with CD
What is the screening test for coeliac disease?

How diagnose?
IgA antibody to TTG

check IgA too and if it's low do IgG TTG

Dx: duodenal Bx
How do you figure out the osmotic gap in stool fluid?

What do the results mean?
serum osmolality = 280

280 - 2([Na] + [K])

>100 = osmotic diarrhoea
<50 = secretory diarrhoea
4 things that cause osmotic diarrhoa
1. monosaccarides
2. disaccarides
3. sorbitol
4. MgCl2
What in the Hx leads you to thinking of osmotic diarrhoea?
diarrhoea ceases when feeds are ceased
Monosaccharide malabsorption is rare and is _____ _________
life threatening
Congenital monosaccharide malabsorption:
What can't be absorbed and why?
glucose/galactose
mutations in SGLT1 (recessive inheritance)
Secretory diarrhoea: due to ____________ ____________ or ______________
(rarely due to congenital defects in electrolyte transporters)
mucosal damage
inflammation
Two broad classification of bloody diarrhoea
1. infection
2. IBD
Presence of leukocytes on stool microscopy indicates _______________-
colitis
Why do you get diarrhoea in IBD?
inflamed colonic mucosa --> malabsorption of fluid and electrolytes
How do you measure protein lost in the stool (protein-losing enteropathy)?
stool alpha 1 antitrypsin levels
What is the main cause of acute gastroenteritis?
viruses (70%)
(bacterial = 15%)
Severity of dehydration:
Dry mucous membranes
Tachy
Abno resp pattern
Sunken eyes
Moderate (5%)
Severity of dehydration:
dry mucous membranes
mottled
cool limbs
slow cap refill
tachy
Severe (10%)
Severity of dehydration:
slow cap refill
cool limbs
mottled
tachy
thready peripheral pulses
Severe: SHOCK
Severity of dehydration:
reduced UO
thirst
dry mucous membranes
mild tachy
Mild (3%)
When in dehydration would you do Ix?
What would you order?
in mod-severe

EUCS (essential in every child having IV fluid), BSLs
FBC
stool micro/culture/virology
Why do rehydration drinks work?
glucose stimulates intestinal sodium transporter
How do you write normal saline and half normal saline on fluid chart?
0.9% sodium chloride (or normal saline)+ 5% glucose

0.45% sodium chloride + 5% glucose
When giving reydration fluid, assume ___% dehydration
5
Calculate fluids for 24h but order for first ______hrs --> r/v
What else do you check at this time?
6-8

repeat EUCs
daily weight
Maintenance fluid regimen
100mL/kg for first 10kg
50mL/kg for second 10kg
20mL/kg for subsequent kg

this is mL/24h
Which fluids do you give for:
Mild dehydration?
oral fluids
Which fluids do you give for:
moderate dehydration?
IV/NG fluids:
rehydration
and
maintenance
Which fluids do you give for:
severe dehydration?
IV fluids
Bolus
plus
rehydration
and
maintenance
Calculation for rehydration fluids
%dehydration (i.e 5) x weight (kg) x 10
over 24h

0.9% normal saline

(assume 5% dehydration)
Bolus calculation
10-20mL/kg of 0.9% sodium chloride or Hartmann's
How much potassium do you add to fluids?
20mmol for each 1000mL bag of fluid

(3mmol/kg/day)

This is sufficient if inital serum potassium was normal and when urine is being passed
How do you give fluids if the child is HYPERnatraemic?
Normal saline or N/2 + 2.5% glucose OVER 48 HOURS
After you're r/v IV fluids at 6-8h, if not improving, then ___________, if improving then _________________
consult

commence oral intake
Should you continue to breast feed during gastroenteritis?
yes
Do you give antidiarrhoeal/anti-emetics to kids with gastro?
no
What are the disaccharides? (3)
lactose, sucrose, maltose
What sort of diarrhoea is cholera?
secretory
Following exposure to shiga toxin E.Coli, presenting with haemolysis and renal failure.
Dx?
Haemolytic Uraemic Syndrome
What does pulsus paradoxus show?
severe obstruction in resp tract
What is the most common precipitator of asthma?
rhinovirus
Which organism causes bronchiolitis most commonly?
RSV
Which organism causes croup most commonly?
parainfluenza types 1,2,3
What are the 2 most common causes of pneumonia in kids?
Pneumococcus
S. aureus
What is a non-specific cough?

Best Rx?
following a viral infection, cough receptors can take a while to settle down

Rx: Vicks vapour rub on chest and honey
Suppurative lung disease = chronic wet cough = __________________________
What are 4 causes of this?
chronic bronchitis

1. secondary bacterial bronchitis
2. CF
3. primary ciliary dyskinesia
4. X-linked hypoglobulinaemia (Abs are the most important part of the immune system against bacterial infection)
What does a psychogenic cough sound like?
a goose

disappears when asleep or preoccupied
What causes epiglottitis classically?

And now?
Hib

Streps, S. aureus, mycoplasma
What two features are classic of epiglottitis?
1. drooling
2. tripod/sniffing position
What organism causes bacterial trachietis?

S+S (3)
S. aureus

1. high fever
2. brassy cough
3. thick purulent secretions
When does spasmodic croup occur?
evening/night
What do you think of with respiratory distress and stridor in a child in first 2wks of life?
laryngomalacia (floppy airways)
- collapse of supraglottic structures during inspiration
Bronchiolitis is typically in children aged <_______

Which days are the peak?
1

days 3,4,5
What do you see on CXR in bronchiolitis? (2)
hyperinflation
patchy atelectasis
Rx Bronchiolitis
supportive

no steroids
O2 if reqd to keep sats >94%
Signs of bronchiolitis (3)
1. wheezing
2. increased work of breathing
3. fine crackles
Number 1 bacterial cause of pneumonia?
strep pneumoniae
Major pathogen in viral pneumonia?
RSV
Signs of bacterial pneumonia (3)
1. scattered crackles
2. marked diminished breath sounds
3. dullness to percussion
Mode of inheritance of CF
autosomal recessive
Best test for Dx CF?
Sweat test
+ve result >60mEq/L
What do you do if sweat test for CF is equivocal?
pancreatic fn: 72h faecal fat collection, stool for trypsin
What organisms do you find in sputum culture in CF Pt? (2)
s. aureus
P. aeruginosa
Next step if you get a +ve newborn screen for CF?
sweat test
or
DNA test
Where is the disease:
Spinal muscular atrophy

What is the acquired disease at this level?
Anterior horn cell

Polio
Where is the disease:
Charcot Marie Tooth

What is the acquired disease at this level?
Peripheral nerve

Guillain Barre
Where is the disease:
Myasthenia gravis

What is the acquired disease at this level?
Neuromuscular junction

this can be both acquired and genetic
Where is the disease:
Duchenne's Muscular Dystrophy

What is the acquired disease at this level?
Muscle

Myositis
The essential Q that must be addressed is whether the infant has central hypotonia or neuromuscular disease

floppy but strong baby suggests _________ cause e.g down syndrome, sepsis, perinatal asphyxia...

floppy but weak suggests _____________ cause
central (more frequent in floppy babies)


peripheral

NB: perinatal hypoxia may be secondary to a neuromuscular disorder
Autosomal dominant = ____% recurrence risk
Autosomal recessive = _____% recurrence risk
X-linked = ______% recurrence risk
50
25
25
How do you tell if a hypotonic infant is from a central CNS cause, or a neuromuscular cause?
central: strong and normal or brisk reflexes

NM: weak and depressed or absent reflexes
primary neuromuscular disorder or anterior horn cell disorder involves proximal or distal muscles?
proximal
peripheral neuropathies involve proximal or distal muscles?
distal
Normally, how long should it take to get up off the floor without using hands?
2 seconds
What is the manoevure called in muscular dystrophy?
What is it an objective measure of?
Gower's
have to use arms to get up

proximal muscle weakness
What happens to calves in muscular dystrophy?
pseudohypertrophy
= fat and collagen

Also see wasting of thigh muscles
What do you think of with:
marked proximal weakness
absent deep tendon reflexes
normal intelligence
tongue fasiculations
bell shaped chest
Spinal Muscle Atrophy

Bell shaped chest cos i/c mm never develop properly so chest wall doesn't develop properly
In SMA, innervation is normal above the ________
tongue
How and when do kids with SMA type I die?
around age 1

resp failure as don't develop resp musculature
EMG (electromyogram) - normal or abnormal in SMA?

Nerve conduction studies?
abnormal: needle into muscle and get electrical equiv of fasciculations due to the fact that the muscle is denervated - see spont contraction of the muscle at the electrical level that correlates with denervation

NCS: Normal. In SMA, this is not where the pathology is cos the nn are normally formed and normally myelinated (the problem is in the anterior horn cell up near spinal cord)
Dx: SMA (2)
1. EMG
2. DNA testing for SMA gene
Sensory deficit: anterior horn cell disease or peripheral neuropathy?
peripheral neuropathy
Mode of inheritance of SMA?
autosomal recessive
Mode of inheritance of Charcot-Marie Tooth?
autosomal dominant
What do you think of with:
clumsy child
pes cavus
claw hand
paraesthesias
Charcot-Marie-Tooth
Dx of Charcot-Marie-Tooth
decreased Nerve Conduction Studies
Sural nerve Bx
Mode of inheritance for DMD?
X-linked recessive
Dx: muscular dystrophy?
Raised CK (the muscle is leaks CK)
defective dystrophin protein from gene Xp21.2
How does DMD present?
progressive muscle weakness
What else can you get in DMD? (4)
Cardiomyopathy
Respiratory insufficiency
Low IQ
Contractures

(dystrophin is expressed in heart and brain)
What can happen in the spine in DMD?
increased lumbar lordosis/scoliosis = compensatory
What does dystrophin do?
gives muscle cell membrane support
without it, loss of structural support and so the fibre is more susceptible to normal wear and tear -
holes in membrane without dystrophin -- calcium influx -- increased inflamm signals -- death of muscle cell and replacement with fibrous tissue
If muscle weakness has a later onset and variable progression, what do you think of?
Becker muscular dystrophy

dystrophin decreased in size and/or amount
What's the difference on DNA analysis between DMD and BMD?
DMD: out of frame deletion or nonsense mutation

BMD: inframe deletion or missense mutation
Mgmt in DMD
multidisciplinary

nocturnal respiration
spinal surgery
STEROIDS (give early and continuously -- unknown why they help)
What is the most common cause of death and disability in kids?
trauma
Injuries in kids are most commonly sustained by what? (3)
1. falls
2. sports
3. MVAs
What is the primary survey?
A- airway and C-spine
B- breathing and ventilation
C- circulation and control of haemorrhage
D- disability and neuro exam
E- exposure and environment
How do you get children into the sniffing position to maintain airway?
put a rolled up towel under the shoulders
When would you intubate?
GCS <8
Cardiac arrest in kids: think ________________ as a cause

_________________ is the commonest cause of cardiac arrest in kids
lungs
hypoventilation

(cf heart in adults usually)
What is the normal RR for infants?
40-60
What do you use to examine circulation in kids?
heart rate

(cf BP)
How do you figure out normal systolic BP in kids?

Diastolic?
= 80 + 2 x age (yrs)

Diastolic = 2/3 x systolic
What is a child's blood volume?
80mL/kg
When giving boluses to improve circulation, what sort of fluid do you give?
crystalloid x 3
then blood
What is normal urine output in kids?
< or = 1 year: 2mL/kg/hour

toddler: 1.5mL/kg/hour

older child: 1mL/kg/hour

(remember 1mL/kg/hour)
What do you do as part of the 'environment' bit of primary survey?
warm with patient with over head warmers or warm fluids
For exposure, it's important to examine everywhere.
What are 5 common sites to miss injuries?
1. scalp
2. neck
3. hands
4. back
5. pernineum
What is involved in the secondary survey? (4)
1. head to foot exam
2. AMPLE Hx
3. imaging
4. think about transfer
What is AMPle Hx?
Allergies
Meds
Past Hx; pregnant
l
e
What must you remember to do in chest trauma?
decompress stomach cos they swallow a lot of air
Spinal cord injury:
Where if -
<8 years old

>8
<8: C1-C3 (think about atlanto-axial dislocation)

>8: C7 injury
Normal physiological values for Neonate:
Pulse
RR
Sys BP
PR: 100-170
RR: 40-60
Sys BP: 50
Normal physiological values for 3-12mo:
Pulse
RR
Sys BP
PR: 100-165
RR: 30-50
Sys BP: 50-90
Normal physiological values for 1-2years:
Pulse
RR
Sys BP
PR: 100-150
RR: 25-35
sys BP: 80-95
Normal physiological values for 3-11 years:
Pulse
RR
Sys BP
PR: 80-120
RR: 20-30
sys BP: 90-110
Normal physiological values for 12-15yrs:
Pulse
RR
Sys BP
PR: 60-100
RR: 15-20
sys BP: 100-120
Developmental dysplasia of the hip:
more common in girls or boys?
girls (6:1)
4 RFs for DDH
1. FHx
2. breech
3. packing ('squashed' in utero due to polyhydramnios)
4. first born
5 clinical features of DDH
Which is the most important clinical sign?
1. asymmetric skin creases
2. shortening
3. Limited abduction in flexion ** most important
4. Barlow's
5. Ortolani's
Imaging for DDH
<6mo: ultrasound
>6mo: xray
What is the best position for baby's hips to be in? (and hence how you brace them in DDH)
flexed
abducted
3 DDx for "Irritable hip"
- hip pain, limp, stiffness

What is most common?
1. transient synovitis * most common
2. septic arthritis
3. Perthes disease
Rx: transient synovitis
rest
ibuprofen
Rx: Perthes disease?
Containment (brace)
50% of kids with Perthes disease will develop _________________ by age 40 and need a total hip replacement

50% of kids with SPFE will develop __________________ by age 50 and need a total hip replacement
osteoarthritis
4 DDx for child with limp (associate with different AGES)
0-2yrs: DDH
2-5yrs: transient synovitis
5-10 yrs: Perthes disease
10-15 yrs: Slipped capital femoral epiphysis
What else do kids get in slipped capital femoral epiphysis?
out-toeing
Rx: SCFE
pinning
Live or non-live:
which one stimulates both B and T cells
Live

Non-live: B >>> T
What do you have to do pre-vaccination?
have anaphylaxis kit ready - 1:1000 adrenaline
CONSENT
What gauge needle do you use to inject immunisations?
23 or 25G
What site for immunisations?
<12mo -> anterolateral thigh
> or = 12mo -> deltoid
What immunisations do you give at: Birth
Hep B
What immunisations do you give at:
2, 4 and 6mo?
Hep B
DTPa (diptheria, tetanus, pertussis)
Hib
Polio
Pneumococcal
Rotavirus
What immunisations do you give at:
12mo?
Hib
Meningococcal
MMR
What immunisations do you give at:
18mo?
Varicella
What immunisations do you give at:
4 years
dTPa
Polio
MMR
What immunisations do you give at:
10-15 years
Hep B
dTPa
Varicella
HPV
Which lobe of the brain is last to mature?
frontal
Organism involved in dental caries
Strep mutans