1
Q
what is eczema
A
itchy, dry inflammatory skin disease
2
Q
what are the 2 type of causes of eczema
A
endogenous - internal
exogenous - external
3
Q
give examples of endogenous eczema
A
- Atopic – ‘genetic barrier dysfunction’
- Seborrheoic – face/scalp – scale associated (babies)
- Discoid – annular/circular patches
- Pomphylx – vesicles affecting palms/soles
- Varicose – oedema/venous insufficiency (rarer in children)
4
Q
give examples of exogenous eczema
A
-Allergic contact dermatitis (sensitised to allergen)
-Irritant contact dermatitis (friction, cold, chemicals e.g acids, alkalis, detergents, solvents) from repetitive motion e.g. hand washing (history)
Photosensitive/photoaggravated eczema – aggrevated by light, obvious pattern – t-shirt cut line
5
Q
what things may eczema flares in children be associated with
A
¥ Infections/viral illness ¥ Environment: central heating, cold air ¥ Pets: if sensitised/allergic ¥ Teething – distribution of face and jaw ¥ Stress ¥ Sometimes no cause for flare found
6
Q
what is the most common type of eczema in kids
A
atopic eczema
7
Q
what leads to the dry inflamed skin in atopic eczema
A
Immune mediated defects in the skin barrier function
8
Q
what is atopy
A
overactive immune response to environmental stimuli
9
Q
what 3 conditions are linked in atopy
A
asthma, eczema, hay fever
10
Q
where does eczema normally start in infants
A
face/ neck then spreads
cheek confused with infantile acne
11
Q
what places are most affected by eczema in older children
A
Flexural pattern predominates -antecubital fossae, popliteal fossae, wrists, hands, ankles
12
Q
what protein is affected in eczema
A
fliggarin
(Filaggrin proteins bind the keratin filaments together, in the epidermis only. Also play a role in producing a natural moisturising factor)
13
Q
what is the treatment for eczema
A
dermal 500
14
Q
what areas are mostly affected by seborrheic dermatitis
A
scalp and face
15
Q
what age range are affected by seborrheic dermatitis
A
3 -12 months
16
Q
what skin commensal is seborrheic dermatitis associated with
A
malassezia
17
Q
what is the treatment of seborrheic dermatitis
A
Emollients, antifungal creams, antifungal shampoos, mild topical steroids (hydrocortisone)
18
Q
describe discoid eczema
A
Scattered annular/circular patches itchy eczema.
Can occur in this pattern as part of atopic eczema or separate entity/diagnosis.
19
Q
why are high dose steroids needed to treat pomphylx eczema
A
hand and foot thicker skin
20
Q
what is pomphylx eczema
A
vesicles of eczema on hands and feet - can be intensely itchy
21
Q
what is varicose eczema associated with
A
venous insufficiency affecting legs
22
Q
which patients may varicose eczema be seen in
A
lymphedema
previous chemotherapy
varicose veins
23
Q
what is the treatment of varicose eczema
A
Emollients, topical steroids, compression stockings (help move fluid)
24
Q
what are some treatments of eczema
A
emollients
topical steroids
25
how much topical steroid should be used to treat eczema
finger tip unit
26
list some topical steroids for eczema
Mild (hydrocortisone) Moderate (Eumovate) 25x Potent (Betnovate) 100x Very potent (dermovate) 600x
27
what are some side effects of topical steroids
skin thinning in prolonged use (not prescribed 3-4 weeks)
Do not use above eumovate on the skin – too thin
Should not cause thinning if used appropriately and with clear instructions
28
how often should you use topical steroids to treat eczema
once daily for 1-2 weeks - then alternate days for a few more days
maintenance - once a week in some areas
29
what things may give you irritant eczema
repeated contact; water and soaps, touching irritant foods; citrus, tomatoes, chemical irritants.
30
what test is done for contact allergens
patch test
| blood test for others
31
what percentage of childhood eczema is mild
80% clear with time
32
what are the immediate symptoms of a food allergy (type 1 hypersensitive)
lip swelling, facial redness/itching, anaphylactoid symptoms
33
what is a type 4 hypersensitivity reaction to food allergy
worsening of eczema 24/48 hours after ingestion
34
what are the 2 ways to test for food allergy
Blood test for specific IgE antibodies to certain foods
| Skin prick testing
35
what are the commonest thing for children to be allergic to
milk/dairy, soy, peanuts, eggs, wheat, fish
| airborne - house dust mite (most common in eczema), pet dander, pollens
36
how does impetigo manifest
pustules and honey coloured crusted erosions
37
what is the causative organism in impetigo
staph aureus
38
how is impetigo treated
Topical antibacterial (fucidin) or Oral antibiotic (flucloxacillin)
39
what virus causes Molluscum contagoisum
Molluscipox virus
40
is Molluscum contagoisum benign
yes - no treatment
41
how does Molluscum contagoisum manifest
Tiny pale, pearly papules, umbilicated centre
42
how is Molluscum contagoisum treated
reassurance
| can use crythrotharpy to activate immune system
43
what cream can be used to treat warts
5% potassium hydroxide
44
what are the most common viral warts in kids
verrucas - on sole of foot
45
what virus causes viral warts
human papilloma virus (HPV)
46
how long do viral warts take to resolve
90% in 24 months
47
what are associated symptoms with viral exanthema
Fever, malaise, headache then rash kicks off (maculopapular over trunk)
48
what virus causes chicken pox
varicella - zoster virus
49
when does chicken pox not give you lifelong immunity
immunocompromised are susceptible at all times
50
how does chicken pox manifest
```
Red papules (small bumps) progressing to vesicles (blisters) often start on the trunk.
Itchy. Associated with viral symptoms
```
51
what is the incubation period for chicken pox
10-21 days
52
how long is chicken pox contagious for
1-2 days before rash appears and until all lesions have crusted over
53
what are rare complications of chicken pox
meningitis
| encephalitis
54
how is chicken pox able to be reactivated as shingles
lays dormant in dorsal root ganglion
55
how does parvovirus manifest (slapped cheek)
Erythematous rash cheeks initially and then also lace like network rash (trunk and limbs) + viral symptoms
56
how long can slapped cheek take to fade
may be 6 weeks - usually self limiting
57
what people are at risk of severe paravirus and why
haemolytic people - virus targets red cells in bone marrow
58
what virus causes hand, foot and mouth
Coxsackie virus A16
| Enterovirus 71
59
how does hand, foot and mouth manifest
clusters of blisters on hands, feet mouth and sometimes bum + viral symptoms
60
how is hand foot and mouth treated
self limiting - supportive treatment
61
what is Orofacial granulomatosis
Lip swelling and fissuring (packing of lips)
| Oral mucosal lesions: ulcers and tags, cobblestone appearance
62
what disease is Orofacial granulomatosis associated with
Crohns disease
| chronic allergy
63
how does erythema nodosum present
red, Painful, erythematous subcutaneous nodules
Over Shins; sometimes other sites
Slow resolution - like bruises, 6-8 weeks
64
what are causes of erythema nodosum
Infections – Streptococcus (throat), Upper respiratory tract (ASO titre/ blood test)
Inflammatory bowel disease
Sarcoidosis – history of cough, serum ACE level
Drugs – OCP, Sulphonamides, Penicillin
Mycobacterial Infections
Idiopathic
65
how is erythema nodosum treated
dermovate
66
what disease is dermatitis herpetiformis linked to
coeliac disease
67
where is dermatitis herpetiformis found
Extensors - Scalp, shoulders, buttocks, elbows and knees
| itchy blisters
68
how is dermatitis herpetiformis investigated
Detailed history, Coeliac screening, Skin biopsy
69
how is dermatitis herpetiformis treated
Emollients
gluten free diet (clear up), topical steroids
dapsone (antibiotic with anti-inflammatory effect on skin)
70
what is associated with urticaria
Associated angioedema (swelling of soft tissue swelling around ribs) (10%)
71
how long may urticaria last for
minutes to 24 hours
72
what is the difference between acute and chronic urticaria
acute <6weeks
| chronic >6 weeks
73
what are some causes of urticaria
Viral infection, Bacterial infection, Food or drug allergy, NSAIDS/
OPIATES (make worse), Vaccinations
74
how is urticaria treated
withdraw triggers
Antihistamines -
Ranitidine, Montelukast
Omalizumab, Ciclosporin (immunosuppressants)
75
what is a complication of Kawasaki disease
can get aneurysms of coronary arteries
| autoimmune
76
what is the incidence of congenital heart disease
8/1000
77
what are the 8 commonest congenital heart defects
```
Ventricular Septal Defect (VSD) – most common
Patent Ductus Arteriosus (PDA)
Atrial Septal Defect (ASD)
Pulmonary Stenosis
Aortic Stenosis
Coarctation of the Aorta
Transposition of Great Arteries (cyanotic heart condition)
Tetralogy of Fallot
```
78
list some drugs that can cause congenital heart disease
Alcohol, Amphetamines, Cocaine, Ecstasy, Phenytoin, Lithium
79
list some infections that can cause congenital heart disease
TORCH (Toxoplasma, others, Rubella, CMV, Herpes)
80
list some maternal causes of congenital heart disease
Diabetes Mellitus, Systemic Lupus Erythematosus
81
when is the most likely period for teratogenic insult
18-60 days
82
what % of congenital heart disease have an underlying chromosomal problem
6-10%
83
what congenital heart defect is associated with trisomy 21 (downs syndrome)
AVSD (atrio-ventricular)
84
what congenital heart defect is associated with trisomy 18 (edwards syndrome)
VSD and PDA
85
what congenital heart defect is associated with trisomy 13 (patau syndrome)
VSD ad ASD
86
what congenital heart defect is associated with turner syndrome
Co-arctation of aorta, bicuspid aortic valve
87
what congenital heart defect is associated with noonan syndrome
Pulmonary Stenosis
88
what congenital heart defect is associated with Williams syndrome
Supravalvular AS
89
what pulse is key to diagnose coarctation of aorta
quiet femoral pulse
90
what investigations may you do for a congenital heart defect
* Blood Pressure
* O2 saturation, arterial BGA (invasive)
* ECG (12 lead, halter monitor, 24hrs, event monitor)
* CXR
* Echocardiogram
* Exercise tests (ECG, sO2)
91
what is dextrocardia
heart on right side
92
what is situs inverses
the major visceral organs are reversed or mirrored from their normal positions
93
BT shunt, balloon valvoplasty, Prostaglandin infusion, pulmonary banding
are all examples of what
operative palliative procedures
94
which L valve open at the start of diastole
mitral
95
which L valve opens at the start of systole
aortic
96
where is best to listen to hear aortic murmurs
upper right and left sternal border
97
where is best to listen to her mitral murmurs
lower left sternal border and apex
98
what percentage of heart murmurs are innocent
70-80%
99
what are the main 4 types of innocent murmurs
Still’s murmur, Pulmonary outflow murmur, Carotid/Brachiocephalic Arterial Bruits
100
in what stage of the cycle are innocent murmurs usually heard
systolic
| venous hum continuous
101
do children with innocent murmurs have other signs of heart disease
no - generally well
102
are innocent murmurs localised
yes - don't radiate far from where loudest
103
how do innocent murmurs change on exercise
louder
104
which is the most common type of innocent murmur
still's murmur
105
describe stills murmur
LV outflow murmur
| Soft systolic; vibratory, musical,”twangy”
106
what age is stills murmur most common in
2-7 years
107
where is stills murmur loudest
apex
| left sternal border
108
when is stills murmur loudest
supine position
| with exercise
109
describe a pulmonary outflow murmur
right ventricular outflow - Soft systolic, vibratory
110
what age is a pulmonary outflow murmur most common in
8-10 years
111
where is a pulmonary outflow murmur heard loudest
Upper left sternal border, well localised, not radiating to back
112
when is a pulmonary outflow murmur loudest
supine position
| with exercise
113
what children is pulmonary outflow murmur most common in
thin / narrow chest
114
which age does Carotid/Brachiocephalic Arterial Bruits affect most
2-10 years
115
where are Carotid/Brachiocephalic Arterial Bruits heard loudest
Supraclavicular, radiates to neck
116
when do Carotid/Brachiocephalic Arterial Bruits get louder
exercise, decreases on turning head or extending neck
117
what age does venous hum occur in
3-8 years
118
describe venous hum
Continuous murmur, sometimes with diastolic accentuation
| Soft, indistinct
119
where is venous hum heard loudest
supraclavicular
120
when can you hear a venous hum
Only in upright position, disappears on lying down or when turning head
121
what are the 3 main types of ventricular septal defect
subaortic
perimembranous
muscular
122
what direction is the shunt in VSD
L--> R (acynotic)
123
where is the hole in a VSD
inter ventricular septum
124
describe the murmur of a VSD
Pansystolic murmur lower left sternal edge, sometimes with thrill
125
what does a pan systolic murmur mean
can hear all through systole and equally as loud
126
why do you hear a diastolic rumble in very large VSDs
relative mitral stenosis
| - fluid overload form LA to LV
127
what do large VSDs lead to eventually
biventricular hypertrophy and pulmonary hypertension
128
what occurs in a VSD to lead to eisenmenger syndrome
PVR increases leads to increased muscle pumping leading to hypertrophy, which increases the pressure in the RA - pulmonary hypertension and leads to R L shunt
129
what location of a VSD is more troublesome
closer to the apex
130
what are signs of heart failure in a child
tachypnoea
tachycardia
hepatomegaly,
failure to thrive
131
how would a large VSD present at birth
signs of heart failure
tachypnoea (pulmonary congestion from LVSD),
tachycardia (reduced SV),
hepatomegaly
failure to thrive (high calorie expenditure).
132
what is a babies biggest exercise
feeding
133
how would you investigate a VSD
longitudinal echocardiogram with coulured Doppler
| Blue and red direction of flow with relation to the ultrasound probe, Yellow and green = turbulence
134
what is the treatment of a VSD
VSD closure - Amplatzer device – plug whole and endocardium grows over
Patch closure – usually pericardium graft
135
what do atrial septal defects usually so
close spontaneously
136
how may an atrial septal defect be diagnosed in adulthood
atrial fibrillation, heart failure or pulmonary hypertension
137
what way is the shunt in atrial septal defects
L > R - acynotic
138
what are signs of a atrial septal defect
Wide fixed splitting of 2nd heart sound, pulmonary flow murmur
139
what congenital heart defect is seen in trisomy 21
low ASD
high VSD
all near valves
140
what are the 4 things of teratology of fallot
1) pulmonary stenosis
2) right ventricular hypertrophy
3) High VSD
4) Overriding aorta – sticks into RV
141
what direction of shunt leads to cyanosis
R > L
142
what shape is the heart on xray in teratology of fallot
boot shaped
143
when can a full correction of teratology of fallot be done
6 months
| before palliative - connect subclavian arteries with pulmonary arteries to create shunt
144
what are symptoms of severe aortic stenosis in children
reduced exercise tolerance, exertional chest pain, syncope
145
describe the murmur of aortic stenosis
Ejection (descend and crescendo) systolic murmur upper right sternal border, radiation into carotids. Suprasternal thrill is pathopneumonic aortic stenosis
146
how would you treat aortic stenosis
Balloon aortic valvuloplasty treats
147
what vessel gives oxygenated blood to the foetus
umbilical vein
148
where is foramen ovale
between RA and LA
149
what causes the ductus arteriousus to close within 24-48 hours
prostaglandin release
150
what is the treatment of PDA in pre-term infants
fluid restriction/ diuretics, prostaglandin inhibitors (Indomethacin, Ibuprofen), surgical ligation
151
what may delayed closure of ductus arteriosus
coarctation of aorta
152
what is a coarctation of the aorta
kink in wall - normally descending arch where ductus arteriosus would enter
153
what is the management of co-arctation of the aorta
Re-open PDA with Prostaglandin E1 or E2 - stabilise
Resection co-arctation with end-to-end anastomosis
Subclavian patch repair (fix hyperplasia of aortic arch)
Balloon Aortoplasty – risk of re-stenosis
154
what occurs in transposition of great arteries that makes the baby cyanotic
aorta comes out of RV and PA comes out of LV so there is no systemic oxygenation occurs
155
what will a baby with transposition of the great arteries need to survive
large VSD, PDA or patent foramen ovale
156
what is given at birth when a baby has transposition of the great arteries
Umbilical venous catheter and prostaglandin infusion at birth to keeps PDA open
157
what procedure is used to treat transposition of the great arteries
Raskind’s atrial septostomy procedure
158
what tests give an actual indication of how the liver is functioning
Coagulation - Prothrombin time (PT)/INR, APTT
Albumin
Bilirubin
(Blood glucose) - glycogen store
(Ammonia) – clearance of toxic metabolites
159
what does a split bilirubin tell the difference between
Direct (conjugated) + Indirect (unconjugated)
160
why are ALT/ AST not that specific to liver disease
also elevated in muscular disease
| alanine aminotransferase/aspartate aminotransferase
161
what is the most common manifestation of paediatric liver disease
jaundice
162
list symptoms/ signs of paediatric liver disease
Growth failure Ascites
Encephalopathy Varices with portal hypertension
Spider naevi Muscle wasting from malnutrition
Splenomegaly with portal hypertension Hypersplenism
Bruising with petechiae Hepatorenal failure
Clubbing Rickets secondary to vit D deficiency
Loss of fat stores secondary to malnutrition Peripheral neuropathy
Hypertonia
163
what is jaundice
yellow discolouration of skin and tissues due to accumulation of bilirubin
Usually most obvious in sclera of eyes
164
at what bilirubin is jaundice usually most visible
total bilirubin >40-50 umol/l
165
is jaundice at < 24 hours old normal
no - always pathological
166
what are causes of jaundice < 24 hours old
sepsis , haemolysis
167
what are some causes of jaundice from 24hrs - 2 weeks (intermediate)
Physiological, Breast milk, Sepsis, Haemolysis
168
what are some causes of jaundice over 2 weeks old (prolonged)
Extrahepatic obstruction, Neonatal hepatitis, Hypothyroidism, Breast milk
169
does physiological jaundice last > 2 weeks (prolonged)
no
170
describe the process of erythrocytes into unconjugated bilirubin
post mature erythrocytes > haemorrhage (in reticuloendothelial system) > biliverdin > unconjugated bilirubin (biliverdin reductase)
171
is unconjugated bilirubin soluble or insoluble
insoluble
172
what enzyme and where is biliverdin converted into unconjugated bilirubin
by biliverdin reductase
| all tissues of body
173
how does unconjugated bilirubin travel to the liver
bound to albumin
174
what enzyme conjugates bilirubin in the liver
UDP glucuranyl transferase
175
is conjugated bilirubin water soluble
yes
176
how ics conjugated bilirubin excreted
by kidneys
bacterial fermantation in gut to be excreted as dark faced
recycled in the entrohepatic circulation
177
is the bilirubin conjugated or not in pre-hepatic jaundice
unconjugated
178
is the bilirubin conjugated or not in post-hepatic jaundice
mostly conjugated
179
is the bilirubin conjugated or not in intra-hepatic jaundice
mixed unconjugates and conjugated
180
is physiological jaundice conjugated or unconjugated
unconjugated
181
why do babies get a physiological jaundice in there first couple of weeks
¥ Shorter RBC life span in infants (80-90 days) – foetal haemoglobin
¥ Relative polycythaemia (Hb 180-200 – breaking down huge amount of red cells and high levels of unconjugated bilirubin)
¥ Relative immaturity of liver function
182
when does a physiological jaundice develop
AFTER the first day of life
183
what kind of jaundice is breast milk jaundice
intrahepatic unconjugated
184
how would you investigate for sepsis in a jaundice baby
urine, blood cultures, torch screen
185
what things may cause haemolysis in a jaundice baby
ABO incompatibility
Rhesus disease
Bruising
Red cell membrane/ enzyme defects
186
how would you investigate for ABO/ rhesus in a jaundice baby
blood group, direct coombs test
187
how would you investigate for red cell membrane defects (spherocytosis) in a jaundice baby
blood film
188
how would you investigate for G6PD red cell enzyme defects in a jaundice baby
G6PD assay
189
name to genetic diseases that may cause jaundice in a baby
Gilberts disease
| Crigler najjar
190
what is crigler- najjar syndrome
absence of G6PD - severe
191
how would you investigate for genetic diseases that may cause jaundice in a baby
genotype/ phenotype
192
what is a sever complication of unconjugaed jaundice
kernicterus
193
how does kernicterus occur
unconjugated bilirubin is fat soluble so can cross the blood brain barrier and deposits in brain (basal ganglia)
194
where in the brain does unconjugated bilirubin deposit to cause kernicterus
basal ganglia
195
what are early signs of kernicterus
encephalopathy – poor feeding, lethargy, seizures
196
what are some late consequences of kernicterus
¥ severe choreoathetoid cerebral palsy, learning difficulties, sensorineural deafness (lifelong)
197
how does phototherapy treat unconjugaed jaundice
visible light converts bilirubin to water soluble photo isomers that can be excreted in the urine
198
what is the treatment of unconjugaed jaundice
phototherapy
199
does the treatment of unconjugated jaundice involve UV light
no - visible (450nm)
200
how will a child need to be treated if they are above the threshold on a growth chart
blood transfusion
201
what is prolonged jaundice
Jaundice persisting beyond 2 weeks of life (3 weeks for preterm infants)
202
is conjugated jaundice in infants normal
no - always investigate
203
what is the most important test in prolonged jaundice
split bilirubin
204
what should you always assess in infants with prolonged jaundice
stool colour
205
what should conjugated jaundice be considered until proven otherwise
biliary atresia
206
how should you investigate for biliary atresia
split bilirubin, stool colour, ultrasound, liver biopsy – special liver centre
207
what colour is the stool is biliary atresia
pale (dark urine)
208
list cause of prolonged jaundice
biliary atresia
choledochal cyst
alagille syndrome
neonatal hepatitis
209
list some causes of neonatal hepatitis
```
Alpha-1 antitrypsin deficiency
Metabolic – galactosaemia, tyrosinaemia
Urea cycle defects
Haemochromatosis
Glycogen storage disorders
Hypothyroidism
Viral hepatitis
Parenteral nutrition – prolonged – after bowel surgery, neo-natal unit
```
210
what is seen in alagille syndrome
Intrahepatic cholestasis, dysmorphism, congenital cardiac disease
211
what is biliary atresia
Congenital fibro-inflammatory disease of bile ducts leading to destruction of extra-hepatic bile ducts with no flow and obstruction (obstructive jaundice)
212
why does biliary atresia need to be identified immediately
rapid progression to liver failure
213
what is the treatment for biliary atresia
kasai portoenterostomy
214
when is a kasai portoenterostomy work best
performed before 60 days (<9 weeks)
215
what should you ask a child complaining of constipation
how often? how hard? is it painful? has there been a change?
| What is normal stool frequency ?
216
what are some signs and symptoms of constipation
• Poor appetite (feel full)
• Irritable
• Lack of energy, look pale
• Abdominal pain or distension
• Withholding or straining – scared of opening their bowels
• Diarrhoea overflow
Only feel batter after they have passed a stool
217
what are some social causes of constipation
```
poor diet - insufficient fluids
excessive milk
not enough plants/ fibre
poor potty training
fear of school toilet
```
218
what medications can cause constipation
opiates, codeine, gaviscon, antispasmodics
219
what are some organic causes of constipation
anal stenosis, anterior anus, Hirschsprung disease, lead poisoning, hypothyroidism
220
describe the viscous cycle of constipation
• Toddler had a hard poo from dehydration/ infection that has caused pain > forms reflex and instinct not to do something painful again ie not touch fire > colon moves stool and sucks out water and salt, arrives in rectum sends message to brain > toddler doesn’t want to so clenches external sphincters to not let out > makes a larger hard stool that is more painful to pass
221
what occurs in mega rectum
back passage gets stretched as they hold internal sphincter open, leading to soiling (overflow diarrhoea)
222
how would you investigate for megarectum
* colonic marker study - take markers Monday, Tuesday, Wednesday and xray on Friday would normally have none but constipated have all in megaractum
* Abdominal examination – sub peritoneal mass, PR
223
what is the dietary treatment of constipation
Increase fibre, fruit, vegetables, fluids
| decrease milk
224
list some ways to reduce psychological causes of constipation
Make going to the toilet a pleasant experience
Correct height – stool or lower toilet/ Not cold/ School toilets
Avoid punitive behaviour from parents
Reward ‘good’ behaviour – retrain brain
General praise/ Star charts – after each meal try push out
225
what drugs can be given to soften the stool and stimulate defecation in constipation
```
osmotic laxatives (lactulose)
stimulant laxatives (senna, picolax)
isotonic laxatives (movicol)
```
226
why do children with mega rectum get a lot of urinary infections and retention
rectum presses on bladder
227
how do you treat impaction
• Empty impacted rectum
Empty colon
Maintain regular stool passage
Slow weaning off treatment
228
when treating constipation what dose of medication and length of treatment os needed
enough to go 3x a day
| duration normally linked to duration of treatment
229
does paediatric IBD affect more boys or girls
boys
230
what is the crohns triad
abdominal pain, diarrhoea, bleeding
231
list some presenting features of crohns disease
abdominal pain, diarrhoea, bleeding but symptoms depend where the disease is
Marked effect on growth as it puts you off eating (anorexia)
Erythema nodosum – raised plaques on shins
232
list some presenting features of ulcerative colitis
bloody diarrhoea usually with mucus, relapsing remitting course
233
name a skin manifestation of IBD
erythema nodosum - raised plaques on shins
234
what is IBDU
IBD unclassified - cant differentiate between UC and crohns – pancolytis (full inflammation), more intensive/ severe
235
what test should you do in IBD to exclude infection
stool culture
236
what lab investigations should you do for IBD
Full blood count & ESR – Anaemia, Thrombocytosis, Raised ESR
Biochemistry - Stool calprotectin, Raised CRP, Low Albumin (losing protein)
Microbiology - No stool pathogens
237
what are radiological investigations for IBD
MRI / MRE water (drink lots of water to open up small bowel)
Barium meal and follow-through (younger kids)
238
if a granuloma is found on mucosal biopsy of the colon, what is the diagnosis
Crohns disease
239
where is ulcerative colitis usually worst
left colon - continuous inflammation
240
where should you always check when doing a colonoscopy for potential IBD
terminal ileum
| ileo-colonoscopy
241
why do children not like being on steroids for IBD
limit growth, make spotty, gain weight, greasy hair
242
what is the medical treatment of IBD
Anti-inflammatory
Immuno-suppression – steroids
Biologics (infliximab)
Immune modulation – azathioprine
243
what is the first line treatment for crohns in kids
polymeric diet - diet of milkshakes for 8 weeks- changes gut flora, improves nutrition
244
what is the end stage in treatment of UC
colectomy
245
how is crohn's treated
Polymeric diet > oral prednisolone > steroid sparing agents – azathioprine/ 6MP or methotrexate > biologics infliximab or adalibumab / surgery
246
how is ulcerative colitis treated
5 ASA (anti- inflammatory) or oral corticosteroids to induce remission > maintain with 5 ASA or immune modulation drug like azathioprine > surgery
247
what is the pre-ejection phase of vomititng
Pallor, Nausea, Tachycardia
248
what is the ejection phase of vomititng
Retch, Vomit
249
what is the post ejection phase of vomititng
weakness, lethargy, floppy, shivering
250
what is the most common thing that stimulates the vomiting centre in children
Infection - UTI in children, meningitis/ encephalitis, cellulitis, sepsis (non specific)
251
list things that stimulate the vomiting centre in children
```
¥ Infection
¥ Enteric pathogens
¥ Intestinal inflammation
¥ Metabolic derangement
¥ Head injury
¥ Visual stimuli
¥ Middle ear stimuli
```
252
what age do children present with pyloric stenosis
4-12 weeks
| boys> girls
253
how do people with pyloric stenosis present
Projectile non-bilious vomiting, Weight loss, Dehydration +/- shock
254
how would you investigate pyloric stenosis
Test feed
Blood gas
Ultrasound – long thickened muscles at pylorus
255
what confirms the diagnosis of pyloric stenosis
metabolic alkalosis on blood gas
256
what are the characteristic electrolyte disturbances of pyloric stenosis
Metabolic alkalosis (↑pH)
Hypochloraemia (↓Cl)
Hypokalaemia (↓K)
257
how is pyloric stenosis managed
Fluid resuscitation
| Surgery – Ramstedts pyloromyotomy – removes pyloris
258
what is seen after a test feed for pyloric stenosis
- Palpation of “olive” tumour - abdomen expands with feed
Visible gastric peristalsis (pushes milk through tight pylorus)
Projectile non bilious vomiting
259
why is there a metabolic alkalosis in pyloric stenosis
loss of hydrochloric acid with vomiting
260
what is the name of the surgical procedure that treats pyloric stenosis
ramstedts pyloromyotomy
261
what is bilous vomiting until proven otherwise
intestinal obstruction (Atresia)
262
give causes of billous vomiting
Intestinal atresia (in newborn babies only) Malrotation +/- volvulus
Intussusception – bowel loops
Ileus – infection slows down
Crohn’s disease with strictures – mainly small bowel
263
what is effortless vomiting almost always due to
gastro-oesophageal reflux
264
what are causes of prolonged reflux
o Cerebral palsy
o Progressive neurological problems
o Oesophageal atresia +/- TOF operated (break in connection to oesophagus, fistula)
o Generalised GI motility problem
265
how may gastro-oesophageal reflux present in children
vomiting, haematemesis
feeding problems/ failure to thrive
apnoea, cough, wheeze
266
what is sandifer's syndrome
association of gastro-oesophageal reflux disease with spastic torticollis and dystonic body movements. Nodding and rotation of the head, neck extension, gurgling sounds, writhing movements of the limbs, and severe hypotonia –these positionings provide relief from discomfort caused by acid reflux
267
what is the usual course of gastro-oesophageal reflux
self limiting - starts at 2 months and gets worse 4-5 months then weans when walking about
268
when would you investigate gastro-oesophageal reflux further
respiratory / feeding problems
269
what is the point of doing a barium swallow
look at anatomy - dysmotility, hiatus hernia, reflux into oesophagus, gastric emptying, strictures
270
how may you investigate gastro-oesophageal reflux
videofluroscopy & barium meal
pH study
Endoscopy
trial of feeding
271
what are disadvantages of a pH study
only detects acid reflux
| may be unpleasant for child
272
what does and oesophageal impedance study tell you
how far acid is coming up
| can pick up other things that aren't acid
273
when is an endoscopy done for gastro-oesophageal reflux
if not resolved at 2 years old
274
what is the best test for oesophagitiis
endoscopy
275
what are disadvantage of doing a trial of feeding to investigate gastro-oesophageal reflux
NG tube and hospitalisation required
276
what are disadvantages of videofluoroscopy and barium meal
may miss reflux
radiation
cause aspiration
277
what feeding advice is given to those with GORD
♣ Thickeners for liquids (keeps it down)
♣ Appropriateness of foods – Texture, Amount
♣ Behavioural programme - Oral stimulation, Removal of aversive stimuli
♣ Feeding position – 45 degrees, head end raised
278
what nutritional support is given to those with GORD
♣ Calorie supplements
♣ Exclusion diet (milk free)
♣ Nasogastric tube
279
what is the medical treatment of GORD
♣ Feed thickener
♣ Gaviscon
♣ Prokinetic drugs
♣ Acid suppressing drugs- H2 receptor blockers – rantitidine, Proton pump inhibitors – omeprazole
280
what are indications for surgery to treat GORD
Failure of medical treatment
Persistent: Failure to thrive
Aspiration – recurrent apnoeic episodes
Oesophagitis – mainly neurodescemities
281
what surgical procedure is done to treat GORD
Nissen fundoplication - wraps fundus around LOS
282
what may be complications of a Nissen fundoplication
bloating, dumping, retching
283
how much water is usually lost in the faeces a day
<200ml
| 9L fluid enters duodenum - 1.5L gets to colon
284
why does the small intestine have such a massive surface area
Mucosal folds + Villi + microvilli
285
what things are secreted by the small intestine
Water for fluidity/enzyme transport/absorption
Ions e.g. duodenal HCO3-
Defence mechanism against pathogens/harmful substances/antigens
286
what is the definition of chronic diarrhoea
4 or more stools per day, For more than 4 weeks
287
how long do acute, persistent and chronic diarrhoea last
acute - <1 weeks
2-4 - persistent
>4 - chronic diarrhoea
288
what motility disturbances may cause chronic diarrhoea
Toddler Diarrhoea
Irritable Bowel Syndrome (functional)
Congenital hyperthyroidism
Chronic intestinal pseudo-obstruction
289
give causes of secretory diarrhoea
Active secretion;
Acute Infective Diarrhoea e.g. cholera
Inflammatory Bowel Disease
290
give causes of osmotic diarrhoea
malabsorption of nutrients ;
Food Allergy
Coeliac Disease
Cystic Fibrosis (fat malabsorption from pancreatic insufficiency)
291
what causes osmotic diarrhoea
Movement of water into the bowel to equilibrate osmotic gradient
Mechanism of action of lactulose/movicol
Generally accompanied by macroscopic and microscopic intestinal injury
292
what predominantly drives the intestinal fluid secretion in secretory diarrhoea
active Cl- secretion via CFTR
293
what is secretory diarrhoea commonly associated with
toxin production from Vibrio cholerae and enterotoxigenic Escherichia coli
294
give causes of inflammatory diarrhoea
- Malabsorption due to intestinal damage
- Secretory effect of cytokines
- Accelerated transit time in response to inflammation
- Protein exudate across inflamed epithelium
295
is stool reducing substance positive in osmotic or secretory diarrhoea
osmotic (digestion of carbs)
296
why does diarrhoea occur in pancreatic disease
lack of lipase and resultant steatorrhoea
297
are electrolytes high in osmotic or secretory diarrhoea
secretory
298
is nocturnal diarrhoea ever normal
no - always pathological
299
how would you determine between secretory and osmotic diarrhoea
stool culture
| appearance
300
what happens to osmotic diarrhoea when food is stopped
also stops
301
what are coeliac people allergic to
gluten - wheat, rye, barley
302
what genes are associated with coeliac disease
HLA DQ2, DQ8
303
give symptoms of coeliac disease
Abdominal bloatedness Diarrhoea
Failure to thrive Short stature
Constipation Tiredness
Dermatitis herpatiformis – vesicular skin rash
304
what skin condition is associated with coeliac disease
dermatitis herpatiformis
305
what serological tests can be done for coeliac disease
Anti-tissue transglutaminase
Anti-endomysial – specific
Anti-gliadin
IgA
306
what test should always be done with coeliac screening
IgA - may be naturally deficient
307
what is the gold standard test for coeliac disease if the screening is positive
duodenal biopsy
308
what are the histological findings of a duodenal biopsy for coeliac disease
Lymphocytic infiltration of surface epithelium
(inflammatory cells)
partial /total villous atrophy,
crypt hyperplasia
309
when can young children be diagnosed coeliac without a biopsy
Symptomatic children
Anti TTG >10 times upper limit of normal
Positive anti endomysial antibodies
HLA DQ2, DQ8 positive
310
what is the treatment for coeliac disease
gluten free diet for life
311
what are complications of coeliac disease
osteoporosis, infertility and Increased risk of rare small bowel lymphoma
312
what leads growth in infants
nutrition
313
what leads growth in children
growth hormone
314
what leads growth in puberty
sex steroid
315
how much weight should a baby gain per week in the first 3 months
200g
316
how much weight should a baby gain per week in the first 3-6 months
150g
317
how much weight should a baby gain per week in the first 6-9 months
100g
318
how much weight should a baby gain per week in the first 9-12 months
75-50g
319
how much weight does a child gain from 1- puberty a year
1kg
320
why do babies need so many calories
Growth (35%)
Physical activity
Thermogenesis
Tissue maintenance
321
why can infants rapidly become malnourished
don't have much reserve
322
how many calories to infants need a day
100kcal and 2g protein/kg/day
323
why is breast feeding better for full term babies
```
well tolerated
less allergenic
Low renal solute load
Ca:PO4, LCP Fatty Acids
Improves cognitive development
Reduces infection - Macrophages and lymphocytes, Interferon, lactoferrin (iron), lysozyme, Bifidus factor
```
324
what are benefits of breast feeding to an infants immune system
develops active immunity
reduces infection
antigen load minimal
325
why is breast feeding not suitable for pre-term babies
need more calories - + fortifiers
326
what are disadvantages of formula feeding
No anti-infection properties
Risk of contamination (food poisoning)
High antigen load
Expensive
327
what are advantages of formula feeding
```
No transition of BBVs/drugs
Doesn’t need mum (working mum)
Accurate feed volumes
Provides Vit K
Less jaundice (breast milk jaundice)
```
328
when should mothers initiate breast feeding (unicef baby friendly 10 steps)
first 30 mins fo birth
329
what is the base of most formula feeds
cows milk
330
what composition of formula milk should be used for 0-6 months
first milk - more like breast
331
why is real cows milk not suitable as main drink for an infant
contains no iron
332
when may a baby receive a specialised formula feed
for cows milk protein allergy
pre term - nutrient – need calories
disease specific
333
what is the most common food allergy in <2s
Cows milk protein allergy
334
what are the majority of cow's milk protein allergy reactions
delayed, non IgE reactions (develop over days) eg vomiting,diarrhoea,abdominal discomfort/ distension, eczema
335
what is the test for cows milk protein allergy
CMP exclusion - should get better
| re challenge after 6 months
336
what are the steps to reintroduce milk into a babies diet to build tolerance
iMAP milk ladder - biscuits/ cookies, muffins, pancakes, cheese, yoghurt, milk
337
what is the first line treatment for cows milk protein allergy
Extensively hydrolysed protein feeds
| second line amino acid
338
are lactose free milks suitable for cows milk protein allergy
no - only free of lactase
339
is lactose intolerance an allergy
no - reduced levels of lactase enzyme
340
what is a downside of soya milk
phytoestrogens
341
when is soya milk indicated
Milk allergy when hydrolysed formulae refused
Vegan families, if not breast fed
Consider for children>1 year still on milk free diet
342
why is rice milk not advised for children < 5
arsenic acid
343
what is the problem with organic milks for babies
not calcium fortified
344
what is weaning and when does it start
Transition from milk to a mixed diet - around 6 months
345
what is a benefit of weaning socially
Encourage tongue and jaw movements in preparation for speech and social interaction
346
what children are at risk of vit D deficiency
dark skin
breast feeding mum not on vit D
Scottish in winter
347
how much calcium fortified milk is needed to meet requirements
400-500ml
348
what should be taken <500ml calcium fortified substitute
Alliance calcium liquid or (if >3y) Calcium softies
| for breast feeding mums Accrete or Cacit D3
349
list some factors that influence height
Age Sex (M>F by 15cm)
Race Nutrition
Parental heights Puberty (early not as tall)
Skeletal maturity (bone age) General health
Chronic disease –inflammatory Specific growth disorders – affect bones/ hormone regulated
Socio-economic status Emotional well-being
350
when is max height reached in boys and girls
end of puberty
girls- 14/15
boys - 16/18
351
how do you measure a baby under 2
length -lying down facing up
| head circumference
352
how do you measure a child over 2
height - stadiometre against wall
353
when may a sitting height be measured
height/ body disproportion e.g achondroplasia, after spinal irradiation
354
how do you measure a childs head circumference
Tape round forehead and occipital prominence (maximal circumference)
355
how is the mid parental height (MPH calculated (potential height for child)
average height of Mum and dad – correct parent of opposite sex i.e. give mum 15 cm if boy)
target centile 10cm +/- MPH
356
what scan do you do to assess a childs bone age
TW 20
| wrist
357
what things can confuse TW 20 scan
osteochondrodysplasic bones
| sever osteopenia
358
what scan can be done to assess growth potential in a child
TW 20
359
what would you ask in the history of a small stature child
* Birth weight and gestation (born small may remain small)
* PMH – chronic disease, intercurrent illnesses
* Family history (height history) /social history/schooling (developmental delay)
* Systematic enquiry – subtle symptoms of growth disorders
* Dysmorphic features – short stature syndromes
* Systemic examination including pubertal assessment – growth potential, crohns, coeliac etc
360
what are some indications to refer a child to a specialist for growth disorders
Extreme short or tall stature (off centiles) – below 0.4th, above 99.6th
Height below target height – disproportional to parents
Abnormal height velocity (crossing centiles)
History of chronic disease
Obvious dysmorphic syndrome
Early/late puberty
361
give non pathological causes of short stature
familial
constitution delay of growth
SGA/ UGR
362
what may kids with IUGR/ SGA benefit from
growth hormone
363
give pathological causes of short stature
Undernutrition chronic illness (JCA, IBD, Coeliac)
Iatrogenic (steroid) Psychological and social deprivation (anorexia)
Hormonal (GHD, hypothyroid) Syndromes (Tuner, Prader-Willi)
364
give features of turners syndrome
wide carrying angle, infantile , shielded chest, wide nipple, webbing of neck, dystrophic/ small nails
365
what are first line investigations for short stature
```
FBC/ ferritin
U&E, CRP, LFT
coelaic/ IgA
hormones - IGF-1, TFT, cortisol, prolactin
karyotype
```
366
what number of the tanner scale is pre pubertal
1
| 2 starts puberty
367
describe the tanner staging of puberty
B 1 to 5 (breast development)
G 1 to 5 (genital development)
PH 1 to 5 (pubic hair) – stimulated by LH and FSH
AH 1 to 3 (axillary hair)
T 2ml to 20ml – prader orchidometer -pubertal >4, adult 12-25ml
368
what is the essential tanner stage of puberty in girls
Breast budding (Tanner Stage B 2)
369
what is the essential tanner stage of puberty in boys
Testicular enlargement (Tanner Stage G2 -T 3- 4 ml)
370
when is puberty considered early and delayed in boys
< 9
| >14
371
when is puberty considered early and delayed in girls
<8
| >13 (rare)
372
which sex are most commonly affected by constitutional delay of growth and puberty
males - FH in dad/ brothers
373
what is the treatment for Constitutional Delay of Growth and Puberty (CDPG)
short course of testosterone
374
what conditions can impair the HPG axis an lead to delayed puberty
septo-optic dysplasia, craniopharyngioma, Kallman’s syndrome
375
what conditions of gonadal dysgenesis may lead to delayed puberty
Turner 45X, Klinefelter 47XXY
376
what causes secondary sexual characteristics to form
sex steroid hormones
377
what imaging must be done in central precocious puberty
MRI - exclude pituitary lesion
378
what is the treatment of central precocious puberty
GnRH agonist
379
what is the cause of Precocious Pseudopuberty
Abnormal sex steroid hormone secretion
380
why must congenital adrenal hyperplasia be excluded in kids
risk of adrenal crisis
381
what are some causes of congenital hypothyroidism
athyreosis/ hypoplastic/ ectopic – no gland or dysfunctional
Dyshormonogenic – dyfucntional thyroxine
382
why is congenital hypothyroidism treated in first 2 weeks
prevent poor development, dysmorphia, severe mental retardation
383
what is the most common cause of acquired hypothyroidism in children
autoimmune hasimotos
384
how may acquired hypothyroidism present in kids
lack of height gain, pubertal delay (or precocity), poor school performance
+ same as adult (constipation, tiredness, skin changes)
385
what centile is an overweight BMI
>85th
386
what skin condition is associated with obesity
acanthosis nigricans (insulin resistance)
387
how would you examine someone for obesity
Weight, Height, Body mass index (BMI)
Waist circumference
Skin folds
388
what drugs may cause obesity in children
insulin, steroids, anti-thyroid, sodium valproate
389
what is the treatment of childhood obesity
diet and exercise
390
list complications of childhood obesity
- Fatty liver disease (non-alcoholic steatohepatitis)
- Gallstones - Reproductive dysfunction (eg, PCOS)
- Nutritional deficiencies - Thromboembolic disease
- Pancreatitis
- Obstructive sleep apnoea
- Gastroesophageal reflux disease
- Orthopaedic problems (slipped capital femoral epiphysis, tibia vara)
- Stress incontinence
- Psychological -Left ventricular hypertrophy
- Atherosclerotic cardiovascular disease - Right-sided heart failure
391
why must DKA be prevented
can lead to permanent neurological disability
392
what are symptoms of diabetes in children(4Ts)
Thirsty, thinner, tired, using toilet more
393
what is a return to bed wetting/ day wetting a red flag for
diabetes
394
how may diabetes present in under 5's
4Ts - Thirsty, thinner, tired, using toilet more
heavier than usual nappies
Blurred vision Candidiasis (oral, vulva) Constipation
Recurring skin infections Irritability, behaviour change
395
list some symptoms of DKA
```
Nausea and vomiting
Abdominal pain
Sweet smelling/ ketotic breath Drowsiness
Rapid, deep sighing respiration
Coma
```
396
how should you test for type 1 diabetes in a child
finger prick capillary glucose test
| If result >11 mmol/l = diabetes
397
what should you do if you suspect a child has T1DM
refer to local specialist - need insulin ASAP
398
how do you calculate the average weight of a child
weight (kg) = 2 x (age +4)
399
what is the average blood volume of a child (mls)
80mls/ kg
400
when is a childs urine output highest
baby - 1ml/ kg/ hour then falls to normal 0.5ml/kg/ hour
401
what is a childs insensible fluid loss a day (breathing, sweating, metabolism etc.)
20mls/ kg/ day
402
how do you calculate a childs systolic BP
80 + ( 2 x age)
| - reaches adult by 20
403
when should you start CPR in a baby
HR <60 - they can't increase their own CO
404
why is referred pain more common in children
struggle to localise
405
what is the problem of paracetamol having a very narrow therapeutic index in kids
can lead to liver failure very quickly
406
why shouldnt codeine be given to kids
20% can’t metabolise so will have no effect and no other analgesias can be mixed, very rarely a patient can have multiple copies of the metabolic enzyme so have a massive response leading to respiratory depression
407
what fluid should be given to a child for resuscitation
20ml/kg bolus 0.9% NaCl
408
what maintenance fluid should be given to children
0.9% NaCl / 5% Dextrose +/- 0.15% KCl
409
what is the 4,2,1, rule for administrating fluid to a child
4ml/kg 1st 10kg, 2ml/kg 2nd 10 kg, 1ml/kg every kg thereafter
e.g. 10 yrs = 2 x (10+4) = 28kg = 40+20+8 = 68mls/hr
410
list some sentinel signs in babies
```
feed refusal
bile vomits
grey colour (tissue perfusion)
Floppy tone
low emperature
```
411
what is acute constant tummy pain more likely to be
peritonitis (peritoneal irritation)
412
what is the dermatome of the umbilicus
T10 - midgut
413
why should you ask a child if they get tummy pain on speed bumps/ movement
rebound pain = peritonitis
414
what question should you always ask a girl with tummy pain
menstraul cycle and related pain
415
what colour is bile
green
416
what is tenesmus
feeling of incomplete evacuation e.g. due to a pelvis full of pus in appendicitis
417
what investigations may you do on a child with abdominal pain
urine
FBC / electrolytes
Xrays - rarely
418
why are X-rays rarely done in children
radiation is cumulative over lifetime
419
what is the diagnostic test for bowel obstruction
Xray
420
what age range is appendicitis most common in
<4
421
what is murphys triad of appendicitis
pain , vomiting, fever
422
which point is tender in appendicitis
mcBurneys - focal
423
what are signs of appendicitis
guarding/ rebound on examination
murphy's triad - pain , vomiting, fever
look unwell
tenderness over McBurney’s point
424
what is the management of appendicitis
laparoscopic removal
425
what are complications of appendicitis
abscess
mass - stuck to bowel
peritonitis
426
give feature of non specific abdominal pain
- short duration – 24hrs - central – peri-umbilical
- constant - not made worse by movement – guarding (speedbump)
- no GT disturbance - no temperature
- site & severity of tenderness vary
427
what is the management for non specific abdominal pain
active observation
428
what does mesenteric adenitis normally follow
follows viral illness - URTI cold, sore throat
| high temperature
429
which location of pneumonia is most common in children
Right lower lobe
430
what are signs that abdominal pain is actually pneumonia
sick
abdomen soft
high CRP
cough - do chest xray
431
which gender are more affected by pyloric stenosis
males 5:1
432
what kind of vomiting is seen in pyloric stenosis
projectile non billows vomititng
| 4-16 week history
433
how would you manage pyloric stenosis
0.45 N Saline/ 5% Dextrose + KCl
0.9% Saline for NG loss
periumbilical pyloromyotomy
434
at what age does malrotation +/- volvulus present
very early - first week
435
what happens to the bowel in malrotation +/- volvulus
bowel twists to cause obstruction
436
how would you investigate malrotation +/- volvulus
upper GI contrast study ASAP
437
how is malrotation +/- volvulus managed
ASAP laparotomy to distort
438
what age does itussusception usually present
6-15 months , rare outside
439
what happens when a baby has a dying spell
baby goes white, floppy, stop breathing for 10s then colic stops and start crying
440
how may intussusception present in a child
colic, dying spells, billows vomiting, poor perfusion (long capillary refill)
441
what type of stool is seen in intussusception
bloody mucous PR (redcurrant jelly stool – mucus and blood)
442
what is though to be the cause of intussusception
Thought to be due to enlarged peyers patches in terminal ileum causing self peristalsis
443
what is the characteristic sign on US of intussusception
target sign (oedematous bowel)
444
what is the management of intussusception
```
pneumostatic reduction (air enema)
laparotomy
```
445
what things are umbilical hernias associated with
low birth weight, down syndrome, hypothyroid, mucopolysaccharide
446
what causes an umbilical hernia
muscles around opening to umbilical cord soft close properly
447
how common are umbilical hernias
1 in 6
448
what % of umbilical hernias resolve spontaneously
90%
449
what are complications of umbilical hernias
obstruction
| strangulation - blood supply cut off
450
when should an umbilical hernia be repaired
by the age of 4 or if complications
451
how can you distinguish an umbilical hernia from a para-umbilical hernia
points down when crying
452
what things increase the size of an umbilical hernia
when laughing, coughing, crying or going to the toilet
453
what causes an epigastric hernia
Defect in linea albea above umbilicus leads to protrusion of peritoneal fat
present form birth - may only notice when thinner
454
when should an epigastric hernia be repaired
not - cosmetic reasons only
455
what causes gastroschisis
congenital problem when gut forms where it doesn’t go back into amniotic cavity
> gut eviscerated and exposed
456
what percentage of gastroschisis are associated with atresia
10% - fatal
457
what is the management of gastroschisis
primary / delay closure
| TPN nutrition
458
what is the survival of gastroschisis
90%
| unless associated with atresia - fatal
459
what may be a complication of short gut
defect nips off small bowel
460
what is exophalos
disturbed embryogenesis leads to external organs covered in membrane
461
what anomalies are associated with exophalos
25% cardiac
25% chromosomal - Trisomy13, 18 (fatal), 21
15% renal, neurological
Beckwith-Weideman syndrome
462
what things are associated with Beckwith-Weideman syndrome
hypoglycaemia, macrogloassia (massive tongue), macrosomia
463
what is the management of exophalos
primary / delayed closure
| Honey dressings
464
what is the outcome of exophalos
poor due to complications
Antenatal 15% survive to birth
post-natal mortality – 25%
465
what is hypospadias
urethral meatus on the ventral aspect of the penis
| Classification – anterior (50%), middle (30%), posterior (20%)
466
what is the management of hypospadias
DO NOT CIRCUMCISE – need skin to reconstruct
| one stage or 2 stages procedure
467
how may urology problems present systemically in children
- fever, vomiting
- failure to thrive
- anaemia
- hypertension
- renal failure
468
how may urology problems present locally in children
- pain
- changes in urine
- abnormal voiding
- mass
- visible abnormalities
469
what is the difference between a hernia and a hydrocele
hernia - groin swelling
| hydrocele - scrotal swelling
470
are hernias common in girls
no - may contain an ovary
471
what type of swelling are premature babies at risk of
hernias - groin swelling
| testis not dropped
472
how common are hernias
affect 2% of boys
473
what type of hernia is more common in children
indirect - through inguinal canal
474
when should hernias be repaired urgently and why
<1 year
| risk of incarceration (get stuck)
475
how would you manage a hernia in a child > 1
elective referral and repair
| growing proportion of hernia orifice to bowel means less likely to get stuck
476
what is the risk of incarcerated testis
compressed blood supply leads to ischaemia and loss of testis
477
are hydroceles symptomatic
no - painless
increase in size when straining, crying
blue in colour
478
what layer of the testis communicates with the scrotum in hydroceles
tunica vaginalis
479
what is the classic sign of a hydrocele
Chinese lantern sign – glows through torch light
480
what is the management of a hydorcele
conservative until 5 yrs of age
| 96% go away
481
what is cryptorchidism
undescended testis - Any testis that cannot be manipulated into the bottom half of the scrotum
482
what is meant by ectopic testis
testis in wrong place – superficial femoral pouch, other side (abnormal descent)
483
why are the testis in the scrotum
permatogenesis enzymes work optimally at 34 degrees. (Heat exchange through scrotal skin contributes to drop in temperature)
484
when do testis normally descend
3rd trimester - any premature baby will always have undescended
485
give indications for orchidopexy
* fertility - 1% loss germs cells / month undescent
* increased risk of malignancy
* susceptible to Trauma – fracture, near femoral neck
* Torsion – twists as not fixed
* cosmetic
486
what is cricumcision
removal of the foreskin
487
what is an absolute indication for circumcisison
Balanitis Xerotica Obliterans (BXO) – white scarring of foreskin
488
what are relative indications for circumcision
Balanoprosthitis (inflammation of skin)
Religious - at 6 months
UTI - if grossly dilated urinary tract with kidney damage
489
what are disadvantages of circumcision
painful
bleeding meatal stenosis – urinary meatus narrows
Fistula cosmetic - look different
490
what are suggested advantages of circumcision
prevents UTI
Decreases malignancy
sexual enhancement - ↑nerve endings in prepuce
decrease of AIDS / STD’s
491
describe the grades of vesicle uteric reflux
1 - ureter only
2 – kidneys but not dilated
3+ 4 – dilated kidneys
5- massive dilation of kidneys, poor function, grossly abnormal urinary tract
492
how is vesico uteric reflux managed
conservative - voiding advice, constipation, fluids
antibiotic prophylaxis - Trimethoprim (2mg/kg nocte)
until age 4
STING procedure - mild/moderate with symptoms
ureteric re-implantation
493
what is torsion appendix testis
embryological remnant of girl bits, can twist
494
what is torsion testis
twisting of testis - can lose blood supply
495
what is the differential diagnosis of painful scrotum
torsion testis
torsion appendix testis
epididymitis/ orchitis
rare - trauma, haematocele, incarcerated inguinal hernia
496
how long after testicular torsion till the blood supply is lost
6-8 hours
497
what is the blue dot sign (ischaemia through skin) a sign of
torson appendix
498
what age does torsion testis usually happen in
older kids
| torsion appendix in younger
499
what age does torsion appendix testis usually happen in
younger children
500
what should you do if you are in doubt of the cause of an acute painful scrotum
open to explore - can't miss torsion
501
what are complications of UTI in kids
renal scarring, reflux nephropathy
chronic renal failure
hypertension
502
who should you investigate UTI's in
• all <6/12 months, atypical infection, odd symptoms, recurrent
pure growth bacteria > 105 – even if low bacteria and symptoms take seriously
Pyuria – pus cells
systemic upset - fever, vomiting
503
how can you investigate UTI's in kids
US scan - anatomy
renography
o 99mTc MAG3 (drainage, function, reflux)
o 99mTc DMSA (function, scarring)
micturating cystourethrogram (MCUG)- demonstrate reflux
504
when does neurodevelopment stop
2 years
505
what must you work out form the neurological history in a child about the symptoms
if they are static (arrest in development)) or slowly progressive
506
what percentage of children form 10-17 suffer migraines
7.7%
507
what percentage of children doe epilepsy affect
0.7%
508
what is the 2nd most common cancer in children
brain tumours
509
what type of headache duration are most likely to have underlying pathology
isolated acute
| chronic progressive - get worse every day
510
what should you ask a child about their headache
```
Any warning?
Location?
Severity?
Duration?
Frequency?
```
511
why do you always do fundoscopy in someone with a headache
look for papilloedema
512
where would you listen for a cranial bruit
side of skull - temporal region
| AV fistula, aneurysm
513
how are migrainea and tension type headaches diagnosed
clinically on history/ exam
514
what associated symptoms ay be seen in a child with a migraine
abdominal pain, nausea, vomiting
| pallor
515
what symptoms may be felt before/ after a migraine
Visual disturbance, paresthesia, weakness
aggravated by bright light (photophobia)
(visual, sensory, motor aura)
516
what things may aggravate a migraine
bright light/ noise
| fatigue/ stress
517
what is the mamangement of an acute migraine
effective pain relief
triptans
sleep/ rest. dark room may help
518
if a child has migraines at least once a week what medication could they be put on
Pizotifen, Propranolol, Amitryptyline, Topiramate, Valproate
519
what is the difference in the pain of a migraine and tension type headache
migraine - hemicranial (one side)
| tension - diffuse, symmetrical all over (tight band), present all the time
520
is there a strong family history of migraines
yes
521
what is the management of an acute tension type headache
simple analgesia
522
what is the prevention of tension type headaches
amitryptilline - discourage analgesics
| Attention to underlying chronic physical, psychological or emotional problems
523
what is waking up from sleep with a headache a sign of
raised ICP
524
what is the treatment of analgesic overuse headaches
```
stop taking
(Headache is back before allowed to use another dose)
```
525
what associated features with a headache would require further neuroimaging
Features of cerebellar dysfunction – poor co-ordination, nystagmus,
Features of raised intracranial pressure
New focal neurological deficit eg. new squint
Seizures, esp focal
Personality change
526
what is a convulsion
Seizure where there is prominent motor activity
527
what is an epileptic seizure
an electrical phenomenon coming from brain
An abnormal excessive hyper synchronous discharge from a group of (cortical) neurons
It may have clinical manifestations – depending on where discharge coming from
Paroxysmal change in motor, sensory or cognitive function
528
what is epilepsy
A tendency to recurrent, unprovoked (spontaneous) epileptic seizures – at least 2 for diagnosis
529
what chemical imbalances trigger seizures
```
Decreased inhibition (gama-amino-butyric acid, GABA)
Excessive excitation (glutamate and aspartate)
Lead to excessive influx of Na and Ca ions - produces an electrical current
```
530
what test can be done to measurlthe current of a seizure
EEG - Electroencephalogram
| measure summation of a multitude of electrical potentials that result in depolarisation of neurones
531
what is the difference between a focal and generalised seizure
generalsied - affect all brain
| focal - restricted to one hemisphere
532
are most epilepsies focal or generalised
generalised
533
what is the commonest cause of an acute symptomatic seizure in childhood
```
febrile convulsion
(associated with fever but without evidence of intracranial infection or defined cause for the seizure)
```
534
what age do febrile convulsions occur at
3 months - 5 years
535
how would you investigate a seizure
```
history
video recording if possible
ECG
EEG
MRI brain
genetics - familial
```
536
what single gene disorder is associated with seizures
tuberous sclerosis
537
what is the difference between an interictal and ictal EEG
interictal - not having a seizure - limited value in diagnosing epilepsy
538
what is the role of Anti-epileptic drugs
control seizures, not cure epilepsy
539
what is the fist line anti-epileptic for children with generalised seizures
sodium valproate
| not child bearing age
540
what is the fist line anti-epileptic for children with focal seizures
carbamazepine
541
what surgical procedure can be done in the management of epilepsy
vagal nerve stimulator – implantable device that generates electrical impulses, under L clavicle wired to L vagus to break epilepsy cycle, family has magnet to use
Epilepsy surgery - remove focal part
542
what is a reflex anoxic seizure
due to vagally mediated bradycardia
e.g. child hit arm against wall very painful vagally mediated pain causes bradycardia (always triggered by pain, fright, upset)
common in toddlers
543
what is the condition where children have psychogenic seizures
non epileptic attack disorder (NAED)
544
when would you suspect a neuromuscular disorder in a child
```
floppy
paucity of limb movements
weak pelvic girdle
delayed motor milestones
frequent falls
myopathic facies
myotonia
foot drop
belly sticks out - poor abdominal s
```
545
what is GOwers sign
ask child to lie flat and get them to stand up without any aids - will roll onto front
546
what are pes caves an hammer toes a sign of
charcot marie tooth disease
547
what things are seen in myopathic facies
droopy eyelids (ptosis), can’t close mouth, poor head control
548
where do neuromuscular disorders affect
Muscle
Neuromuscular junction-
Nerve -
Anterior Horn Cell
549
what is the commonest neuromuscular disorder in children
duchenne muscular dystrophy
550
what gene is mutated in duchenne muscular dystrophy
Xp21, dystrophin gene
| X linked
551
how is duchenne muscular dystrophy inherited
X linked
552
what are signs of duchenne muscular dystrophy
Waddling gait, calf hypertrophy (replaced by fat), Gower’s sign positive
553
why are creatine kinase levels so high in duchenne muscular dystrophy
muscle breakdown
554
what is the main treatment of duchenne muscular dystrophy
steroids
555
what is a complication of duchenne muscular dystrophy
cardiomyopathy
| respiratory in teens
556
do myopathies or neuropathies have contracture
myopahty
557
do reflexes last longer in myopathy air neuropathy
myopathy
558
what do anterior horn cell pathologies/ spinal muscular atrophy lad to in adults
motor neuron disease
559
what are the most common cancers in children
leukaemia 33%
brain tumour 25%
lymphoma
560
how are children's cancer classed
Based on tumour morphology and (primary site)
561
what do children with falcon anaemia have a high change of getting
aplastic leukaemia
| get adult cancers in youth
562
what is Li-Fraumeni Familial Cancer Syndrome
AD mutation in p53 – leukaemia, sarcoma, brain tumour, adrenal cortex tumour (50% of cancer by 30, 90% by 70)
563
what canacers are children with beckwith weidmann syndrome screened for
hepatoglastoma
| WInn's tumour
564
name to infections that can drive cancers in kids
HPV - cervical
| EBV - lymphoma
565
which presenting symptoms need a same day referral to a paediatric oncologist
unexplained petechiae, hepatosplenomegaly
566
which presenting symptoms need referral to a paediatric oncologist
```
repeat attendance, same problem, no clear diagnosis
new neuro symptoms, abdo mass
rest pain (night), back pain and unexplained lump lymphadenopathy
```
567
what are signs of brain tumours in pre school kids
```
persistent/ recurrent vomiting
abnormal balance/ walking
abnormal eye movements
behaviour change
fits/ seizure
abnormal head position
```
568
what are signs of brain tumours in children 5-11
```
persistent/ recurrent vomiting
persisten/ recurrent headache
abnormal balance/ walking
abnormal eye movements
behaviour change
fits/ seizure
abnormal head position
```
569
what are signs of brain tumours in young adults 12-18
```
persistent/ recurrent vomiting
persisten/ recurrent headache
abnormal balance/ walking
abnormal eye movements/ blurred/ double vision
behaviour change
fits/ seizure
delayed/ arrested puberty
```
570
list 4 oncological emergencies
* Sepsis / febrile neutropenia
* Spinal cord compression
* Mediastinal mass
* Tumour lysis syndrome
571
how may you inestigate a cancer in a child
MRI - no radiation
biopsy
tumour markers
572
what hormone does testicular cancer secrete
BHCG
573
what are acute SE of chemotherapy
Hair loss, Nausea & vomiting, Mucositis, Diarrhoea / constipation
Bone marrow suppression – anaemia, bleeding, infection
574
what are chronic side effects of radiotherapy
Fibrosis / scarring, Second cancer, Reduced fertility
575
what are chronic side effects of chemotherapy
Organ impairment – kidneys, heart, nerves, ears
Reduced fertility
Second cancer
576
what are acute side effects of radiotherapy
Lethargy, Skin irritation, Swelling
| Organ inflammation – bowel, lungs
577
which oncology patients are at risk of sepsis
ANC < 0.5 x 109
Indwelling catheter – route for bacteria
Mucosal inflammation/ dairrhoea
High dose chemo / SCT
578
what bacteria commonly cause sepsis in cancer patients
```
Pseudomonas aeruginosa
Enterobacteriaciae eg E coli, Klebsiella
Streptococcus pneumoniae
Enterococci
Staphylococcus
Fungi eg. Candida, Aspergillus
```
579
how may a child with cancer present with sepsis
Fever (or low temp) –
Rigors
Drowsiness
Shock - Tachycardia, tachypnoea, hypotension, prolonged capillary refill time, reduced UO, metabolic acidosis
580
how may you investigate for sepsis in kids with cancer
```
Blood culture, FBC, coag, UE, LFTs, CRP, lactacte
CXR (respiratory symptoms)
Other - Urine microscopy / culture
Throat swab
Sputum culture / BAL
LP
Viral PCRs
CT / USS (pus in liver/ badder)
```
581
what is the management of sepsis in cancer patients
ABC – oxygen, fluids (IV access)
Broad spectrum antibiotics – IV tazicin
Inotropes
PICU – paediatric intensive care unit
582
what antibiotic is given to kids with cancer who become septic
IV tazicin
583
why is raised ICP not common in babies
fontanelles aren't fused
584
what is tumour lysis syndrome
Metabolic derangement causing rapid death of Tumour Cells
| Release of intracellular contents into plasma – high K , Ph and lead to death from arrhythmia
585
what caner is tumour lysis syndrome common in
burkett's lymphoma
586
what are clinical features of tumour lysis syndrome
high K, high rate, high phosphate, low calcium
acute renal failure
arrhythmia
587
how is tumour lysis syndrome managed
```
Avoidance – many fluids
ECG Monitoring
Hyperhydrate-2.5l/m2
QDS electrolytes
Diuresis
Never give potassium
```
588
how may you treat hyperkalaemia in kids
¥ Ca Resonium
¥ Salbutamol
¥ Insulin
589
what are common causes of superior vena cava syndrome
lymphoma
| neurobalstoma, germ cell tumour, thrombosis
590
how may Superior Vena Cava syndrome present
facial, neck and upper thoracic plethora, oedema, cyanosis, distended veins, ill, anxious, reduced GCS, watershed mark
591
how may Superior Mediastinal Syndrome present
dyspnoea, tachypnoea, cough, wheeze, stridor, orthopnoea
592
how is SVCS/ SMS managed
keep upright/ calm
urgent biopsy
chemotherapy
steroids
593
what are early signs of raised ICP in children
early morning headache/vomiting
tense fontanelle
increasing head circumference
594
what are late signs of raised ICP in children
```
constant headache papilloedema
diplopia (VI palsy) – double vision
Loss of upgaze
neck stiffness status epilepticus,
reduced GCS - drowsy
Cushings triad (low HR, high BP, low RR)
```
595
which imaging is best for raised ICP
MRI
596
what is the management of raised ICP
dexamethasone
| reduces swelling and increases CSF flow
597
what surgical procedures can be done to treat raised ICP
```
Ventriculostomy – hole in membrane at base of 3rd ventricle with endoscope
EV Drain (temporary) – release oedema
VP shunt – ventricles to tummy
```
598
what is cushings triad (raised ICP)
low HR, high BP, low RR)
599
what cancers common cause spinal cord compression
Ewing's (vertebral body)
Medulloblastoma (back of head)
neuroblastoma
germ cell tumour
600
how is spinal cord compression as a complication of cancer managed
Urgent MRI
Start dexamethasone urgently to reduce peri-tumour oedema
Definitive treatment with chemotherapy is appropriate when rapid response is expected
601
how may spinal cord compression as a complication of cancer present
```
Symptoms vary with level
weakness (90 %)
pain (55-95 %)
sensory abnormality (10-55%)
sphincter disturbance (10-35%
```
602
what organ separates URTI and LRTI
larynx
603
is the respiratory tract sterile
no
604
are URTI's self limiting
yes - 99%
605
do you give antibiotics for URTI
no - mostly viral
| do more harm than good
606
how many episodes of rhinitis may a child get a year
5-10 - normally winter months
607
how long does rhinitis normally last
11-14 days
| self limiting
608
how may a viral illness lead to a more severe virus
breach in epithelium making commensal bacteria infective
| pneumonia, bronchiolitis, meningitis, septicaemia
609
how long does otitis media last
3-7 days
610
how may otitis media appear on otoscope
red
dorme not transparent
painful
611
what is the treatment of otitis media
spontaneous rupture of drum
612
what drug should be given in otitis media
analgesia
613
how long does tonsillitis last
2-5 days
614
how do you differentiate between viral and bacterial tonsillitis
throat swab
615
what drug should you not give in tonislitis and why
amoxicillin - rash with EBV
616
how would you treat bacterial tonsillitis
10 days penicillin
617
what organism causes croup
para'flu 1
618
how long does croup last
2-3 days
619
why is epiglottis rare
vaccinated against H. influenzae Type B
620
what is the biggest difference in the presentation of croup and epiglottis
croup the child is well, epiglottis the child is very ill
621
how may a child with group present
Coryza++, stridor, hoarse voice, “barking” cough (airway obstruction)
622
how may a child with epiglottis present
septic, Stridor, drooling
623
what is the treatment of croup
oral dexamethasone
624
what is the treatment of epiglottis
intubation and IV antibiotics
625
what organism causes epiglottis
H. influenzae Type B
626
what is the main management for LRTI
oxygenation, hydration, nutrition
627
what bacterial agents commonly cause LRTI
Strep pneumoniae, Haemophilus influenzae, Moraxella catarrhalis, Mycoplasma pneumoniae, Chlamydia pneumoniae
628
what viral agents commonly cause LRTI
RSV, parainfluenza III, influenza A and B, adenovirus, rhinovirus
629
how may bronchitis present
loose rattly cough. Lasts over 2 weeks.
| Post-tussive vomit - “glut” (mucous), but chest free of wheeze/creps
630
what is the most common organism causing bronchitis
haemophillus influenza
| pneumococcus
631
does bronchitis need treatment
no -self limiting
632
what is the mechansim of bronchitis
virus disturbs mucociliary escalator clearance, increasing cough and rattle until cleared
633
what are red flags for bronchiectasis not bronchitis
Age <6 mo, >4yr Static weight
Disrupts child’s life Associated SOB (when not coughing)
Acute admission Other co-morbidities (neuro/gastro)
634
what % of infants get bronchiolitis
30-40%
635
how does bronchiolitis present
One off episode - Nasal stuffiness, tachypnoea, poor feeding, Crackles +/- wheeze
636
what is the treatment for bronchiolitis
Maximal observation, minimal intervention (if oxygenation, nutrition and hydration ok)
637
how long may bronchiolitis last for
7-14 days
| noticed at 2-5 days at peak
638
what organism commonly cause bronchiolitis
usually RSV
| parable III, HMPV
639
is a child has a wheeze is it viral or bacterial
viral
640
what is a LRTI defined as
48 hrs, fever (>38.5oC), SOB, cough, grunting
641
what are symptoms/ signs of LRTI
fever (>38.5oC), SOB, cough, grunting
| ¥ Reduced or bronchial breath sounds
642
when can you call a LRTI pneumonia
signs are focal (in one area)
Crepitations
High fever
643
what is the first line treatment for pneumonia in kids
oral amoxicillin
| macrolide 2nd
644
how would you treat a milk LRTI
not - review if gets worse
645
how does pertussis present
coughing fits, vomiting and colour change.
| >2 weeks of cough
646
how would you treat bilateral otitis media in an infant
oral amoxicillin
647
what are the symptoms of asthma
Chronic wheeze
cough - dry, nocturnal, exertional
SOB- at rest
648
what things may trigger asthma in kids
URTI, exercise, allergen, cold weather, etc
649
what is a cardinal feature of asthma that separates it form COPD
reversible/ variable
650
what are the 3 cardinal features of asthma
chronic wheeze
reversible/ variable
responds to treatment
651
what are some potential causes of asthma
Genes (50-80% risk) - Inherently abnormal lungs, poor lung function
Early onset atopy
Late exposures – rhinovirus, exercise, smoking
Occupational – baker, latex etc.
652
can you grow out of asthma
yes
653
if you have asthma how much more likely are you to get COPD
5x
654
what is the differential diagnosis of asthma in <5s
```
Congenital airway abnormality
CF
PCD – primary ciliary dyskinesia
Bronchitis
Foreign body
(unlikely)
```
655
what is the differential diagnosis of asthma in >5s
Dysfunctional breathing (psychological)
Vocal cord dysfunction
Habitual cough
Pertissus
656
what is the treatemtn of asthma
Watchful waiting if no QoL implications of symptoms
Low dose ICS for 2 months
Review after 2 months – step up treatment/ give inhaler holiday
657
what can parents do to help their childs asthma
stop tobacco smoke exposure, remove environmental triggers (cat, dog etc.)
658
what drug is given in adult asthma but not kids
LAMA
659
what are the goals of asthma treatment
```
“minimal” symptoms during day and night
minimal need for reliever medication
no attacks (exacerbations)
no limitation of physical activity
normal lung function (in practical terms FEV1 and/or PEF >80% predicted or best)
Increase dose if not effective.
```
660
what survey can me done to measure control of asthma
```
SANE questionnaire
Short acting beta agonist/week
Absence school/nursery
Nocturnal symptoms/week
Excertional symptoms/week
```
661
what is the goal of well controlled asthma
no more than 1-night wakening to asthma, use blue less than 3 days a week.
662
what classes of medications should be used to treat asthma
```
¥ Short acting beta agonists
¥ Inhaled corticosteroids (ICS)
¥ Long acting beta agonists*
¥ Leukotriene receptor antagonists*
¥ Theophyllines*
¥ Oral steroids
```
663
what is the first line treatment of asthma
low dose ICS - review after 2 months for response, may step down to B2 agonist
664
what is the second stage of asthma treatment after diagnosis
B2 agonist
| + regular preventer - ICS
665
what are adverse effects of ICS
height suppression (0.5-1cm), oral candidiasis (brush teeth), adrenocortical suppression (stronger purple inhaler)
666
what is the 3rd stage of asthma treatment in kids
Add on LABA – do not use without ICS. Use as fixed dose inhaler.
Or LTRA - Montelukast only, Rule of thirds for efficiency
667
what is the 4th stage of asthma treatment in kids
refer - high dose ICS
668
what are the 2 types of delivery systems used for inhalers
MDI (metered dose inhaler)/ spacer
| dry powder device
669
what is the ling deposition when using a delivery system with an inhaler
20%
| 5% without
670
what age can use a dry powder device
licensed in over 5s
| struggle under 8 - try in girls first
671
does losing weight help treat asthma
no but improves general health
672
what gut disease may a painful joint be assoiciated with
IBD
673
what blood disorder may a painful joint be associated with
haemophilia
674
what are red flag symptoms of joint pain
Fever
Malaise/lethargy
Morning joint stiffness or pain
Night pain refractory to simple analgesia and symptomatic during the daytime
675
what are red flag signs of joint pain
• Joint swelling
• Bony tenderness to palpation
• Muscle weakness
Fall in height or weight growth curve
676
how may you investigate a painful joint
```
FBC including ESR/ CRP
blood culture
Xray
US - effusion of septic arthritis
MRI - inflammation sensitive
Bone scan - osteomyelitis
CT
```
677
after inflammation how long does CRP and ESR take to rise
ESR - 48 hours
| CRP - 6 hours
678
what is the most common chronic rheumatological disease in children
JIA
679
what age does JIA affect
<16
680
which sex are more affected by JIA
female 3:1
681
what pro inflammatory markers are seen in JIA
TNF, interleukin 1 & 2. (cause presence of antibodies – ANA, RF)
682
give the different classifications of JIA
```
oligoarthritic < 4 joint
polyarticular > 5 joints
systemic
enthesitis
psoriatic
```
683
when are children most affected by oligoarthritic JIA
girls 1-5
684
what eye sign is seen in ANA positive oligoarthritis JIA
uveitis
685
how do young girls present with ologiarthritis JIA
large joints affects - knees, ankles, hands, feet and hips
NOT HIP
uveitis
+ve ANA
686
if a child with oligoarthritis JIA presents late are they likely to be ANA -ve or +VE
-ve
(boys > 8)
hip involvement
687
how many joints are affected in oligoarthritic JIA
< 4
688
how many joints are affected in polyarticular JIA
>5 joints
689
what is the difference between seropositive and seronegative polyarticular arthritis
```
-ve = RF -ve (20-30%) <5s
+ve = RG +ve (5-10%) >8s
```
690
do people with seropositive or seronegative polyarticular JIA have a better or worse prognosis
seropositive
691
what are symptoms of temporomandibular joint involvement in polyarticular JIA
limited bite
| micrognathia
692
are systemic manaifestations seen in polyarticular JIA
rare - fever, slight hepatoslenomegaly, lymphadenopathy, pericarditis, chronic uveitis
693
what joints are most commonly affected in polyarticualr JIA
large, fast growing joints
TMJ
onset insidious
694
what is enthesistis JIA
inflammation of the enthesis along with arthritis
695
what is an enthesis
where tendon joins the bone
696
what features are seen in Enthesitis related JIA
Onset of polyarthritis/ oligoarthritis in a boy > 8 years
HLA B27 positivity
Acute anterior uveitis
Inflammatory spinal pain
Sacroilliac joitn tenderness
Family history of enthesitis – related JIA
697
what features are seen is psoriatic JIA
HLA- B27 positive
Family history of psoriasis
Dactylitis (finger or toe inflammation) - mostly affects small joints of hands
Oncholysis, nail pitting
698
what joints does psoriatic JIA most commonly affect
small joints of hand - dactyltiis
699
what are features seen in still's disease (systemic JIA)
unwell – intermittent fever > 2 weeks (spiking temperature, apyrexial in between), salmon pink erythematous rash, generalised lymphadenopathy, serosisits, hepatomegaly/ splenomegaly
High inflammatory markers
700
what is a key feature of the temperature seen in still's disease
spiking - child is well in between
701
what are symptoms of JIA
Joint Pain
Swelling
Stiffness: Early morning, relieved by movement Babies / infants: crying or irritability
Limp or abnormal gait Regression of milestones in infants
Delayed puberty/ growth Fatigue (autoimmune conditions)
Poor appetite/ weight loss
702
what are systemic symptoms of JIA
Fever, Rash, Lymphadenopathy, Hepatomegaly/splenomegaly, serositis
703
how long must the arthritis last for it to be JIA
6 weeks
704
in JIA, when is the joint most swollen
morning
| relieved by movement
705
what are signs of JIA
Swelling - periarticular soft tissue oedema/intraarticular effusion/ hypertrophy of synovial membrane
Tenosynovitis (swollen tendons)
Joint held in position of maximum comfort (knee fixed flexion)
Range of motion limited at extremes. (Due to inflammation and fluid)
706
what is the differential diagnosis of JIA
Septic arthritis Transient synovitis
Osteomyelitis – infection of bone – Xray/ US will show Rheumatic fever
Connective tissue disorder - Systemic Lupus
Juvenile Dermatomyositis
Associated with IBD
Recurrent haemarthrosis – boys with haemophillia
Growing pain
Leukaemia Neuroblastoma
Primary bone tumour
Perthe's disease Slipped upper femoral epiphysis
Congenital hip dysplasia Fracture
Trauma Referred pain
707
give factors that may cause a poor prognosis in JIA
Active disease at 6 months Polyarticular onset and course
Extended oligoarticular Female
Rh Factor +ve ANA +ve
Persistent raised inflammatory markers
708
what is the test for JIA
isn't one
Consider:
Anti-nuclear antibodies (ANA) HLA-B27
inflammatory markers (CRP, ESR, WBC, platelets) Rheumatoid factor
X-ray/ US
MRI with contrast - synovitis
•Exclude differential diagnosis
709
what is the most important investigation in JIA
MSK examination of all joints
pGALS, pREM
movement restriction
710
what is the non pharmacological management of JIA
physiotherapy - strength & condition, range of movement, hydrotherapy
Occupational therapy, Psychology (counselling for patients and parents)
opthalmology screening
Nutrition – address anemia and osteoporosis
711
what is the drug management for JIA
NSIAD
Intra-articular steroids
DMARD - methotrexate
biologics - anti-TNF , in conjunction
712
what DMARD is used to treat JIA
methotrexate - subcutaneous weekly
713
what drug is used to treat oligoarticualr JIA
Intra-Articular steroids
714
what drug is used to treat polyarticualr JIA
systemic steroids - oral/ IV methylprednisolone
715
what are complications of JIA
Altered growth of limbs – localised disturbance such as a leg length discrpency
Scoliosis
Short stature – poor growth with active inflammation
Joint damage / destruction
Micrognathia – TMJ involvement (seen in polyarticular)
Blindness (untreated uveitis)
Contracture – now rare, treated aggressively
Psychosocial effects of chronic disease
716
what screening do all children with JIA undergo
opthalmology every 3-4 months until 12
| within 6 weeks of diagnosis
717
what type of JIA is uveitis most common in
ANA positive oligo JIA Common in < 5 years, girls
718
what is the treatment of uveitis
Initially topical steroids to reduce inflammation (More severe need systemic steroids)
tend to have poor response to steroids - DMARD and biologics
719
what is SIRS
systemic inflammatory response syndrome
720
what is seen in SIRS
Fever or hypothermia
Tachycardia
Tachypnoea
Leucocytosis or leucocytopaenia
721
what is meant by bacteraemia
bacteria multiplying in the bloodstream
722
which organs fail in sepsis in children
Respiratory failure Renal failure
Neurologic failure Haematological Failure
Liver failure
723
what pathogens are the most responsible for sepsis in neonates <1 month
group B streptococci, eschericha coli
listeria monocytogenes
(colonisation of female genital tract)
724
what pathogens are the most responsible for sepsis in children
streptococcus pneumoniae, meningococci, group A streptococci, staphylococcus aureus
725
describe the pathophysiology of sepsis
LPS, peptoglycans and bacterial components released in blood stream
stimulate endothelium, neutrophils and monocytes to release cytokines.
This leads to microvascular occlusion, and vascular instability
septic combination of coagulopathy, fever, vasodilation and capillary leak
726
give symptoms of sepsis in children
```
¥ Fever or hypothermia
¥ Cold hands/feet, mottled, prolonged capillary refill time
¥ Chills/rigors
¥ Limb pain
¥ Vomiting and/or diarrhoea
¥ Muscles weakness
¥ Muscle/joint aches
¥ Skin rash
¥ Diminished urine output
```
727
what is the recognition tool for children with sepsis
SEPSIS 6
728
what is the management of sepsis in children (Sepsis 6)
1. Give high flow O2
2. Obtain IV access and obtain bloods (blood culture, blood sugar, lactate)
3. Give IV antibiotics – cefotaxime + amoxicillin if neonate
4. Consider fluid resuscitation - 20mls/ kg crystalloid- aim to reverse shock and normalise HR/ BP
5. Consider early inotropic support – adrenaline
6. Involve senior/ specialist help early
729
what age group has the highest prevelance of sepsis
new borns (preterm > term) 1;300
730
what are the most common organisms that cause meningitis in neonates
group B streprococci, eschericha coli
listeria monocytognes
(female genital tract)
731
what are the most common organism that cause meningitis in children
streptococcus pneumoniae, meningococci, haemophilus influenza
732
give symptoms of meningitis in children
```
¥ Nuchal rigidity
¥ Headaches, Photophobia
¥ Diminished consciousness
¥ Focal neurological abnormalities
¥ Seizures – late sign
¥ Meningococcal rash – large scale purpura and bruising
```
733
describe a meningococcal rash
large scale purpura
734
how many meningitis present differently in neonates
Lethargy, Irritability
Bulging fontanelle – pressure around brain increases CSF
‘nappy pain’- stretch meninges
735
how may you investigate meningitis in children
FBC
CSF:
Blood and CSF cultures (antigen testing, PCR) – become positive 24 hours later
Urine culture, skin biopsy culture
blood gas; metabolic acidosis
Imaging: CT-cerebrum – make sure you can do a LP
736
what would you expect to see on a FBC in a child with meningitis
leucocytosis, thrombocytopaenia
CRP; elevated – marker of inflammation
coagulation factors; low levels due to DIC, low glucose
737
what finsigs would you expect to see in the CSF of a child with meningitis
pleocytosis (High WCC) , increased protein level, low glucose (bacteria feeds on glucose)
738
who is given chemoprophylaxis if a child has meningitis
close household contacts
739
what is the antibiotic treatment for meneingits in children and neonates
3rd generation cefalosporins
| + amoxicilline if neonate – covers listeria
740
is streptococcus pneumonia gram positive or negative
Gram-positive Duplo-cocci (Diplococcus pneumoniae)
741
where does streptococcus pneumonia colonise
upper airways - 5-10% adults, 20-40% children
742
how is streptococcus pneumonia transmitted
droplets
743
what are complications of pneumococcal meningitis
brain damage, hearing loss, hydrocephalus
744
is Haemophilus influenza type B gram positive or negative
negative
| encapsulated
745
what is a common predisposing factor for severe bacterial infections in children
viral illnesses
746
why does meningococcus in the bloodstream have such an early mortality
lipooligosachharide -association endotoxin very potent stimulus of immune system
747
what is the mortality of meningococcal disease
5-15% - 50% of deaths in first 12 hrs, 80% within 48 hrs
748
what are common complications of meningococcal disease
Amputation (14%) – DIC, purpura
skin scarring (48%)
Cognitive impairment/epilepsy/hearing loss
749
where is meningococcus usually found in children
nasopharynx
| B and C (X Y becoming more prevalent)
750
what do streptococci and staphylococci have in common
both gram positive cocci
751
what antibiotic is active against streptococci
penicillin
752
what antibiotic is active against staphylococci
flucloxacillin
| resistance - need glycopeptide
753
what antibiotic should you use if you are unsure if the infection is staphylococci or streptococci
flucloxacillin
754
what are the main symptoms seen in toxic shock syndrome in children
warm
| very red rash
755
describe the sequence of scarlet fever
```
Contact and incubation 2-4 days
Malaise, fever, tonsillitis
Couple of days later Start exanthema
Strawberry tongue (characteristic sign)
Squamation of hands and feet (after acute phase- 1-2 weeks later)
```
756
what is a characteristic sign of scarlet fever
strawberry fever
757
when is squamation of the hands and feet seen in a scarlet fever infection
late sign - 1 2 weeks after
758
wat ist he cause of scarlet fever
Group A β-hemolytic streptococci
759
what protects most children < 2 form scarlet fever
maternal IgG antibodies
760
what is the most common ge for a scarlet fever infection
4
| rare <2 and >10
761
what are some complications of scarlet fever
Erysipelas, Cellulitis, Impetigo
Streptococcal toxic shock
Rheumatic fever 0.3-3%
Glomerulonephritis
762
what is the treatment of scarlet fever
penicillin 10 days
763
give features of Staphylococcal scalded skin syndrome (SSSS)
fever, widespread redness, fluid-filled blisters, rupture easily, especially in the skin folds. Superficial skin disappears, looks like burns in a patient
764
give symptoms of kawasaki disease
```
Rash + Fever for at least 5 days and four of the five;
¥ bilateral conjunctival injection
¥ changes of the mucous membranes
¥ cervical lymphadenopathy
¥ polymorphous rash
¥ changes of the extremities
+peripheral oedema
+ peripheral erythema
+ periungual desquamation (under nails)
```
765
describe the pathophysiology of Kawasaki disease
self-limited vasculitis of medium-sized arteries
increased risk siblings and twins (genetic factor)
aetiology unknown but infectious cause suggested
766
what are complications of Kawasaki disease
vasculitis of coronary arteries and kidney arteries
767
what is the treatment of kawasaki disease
High dose Immunoglobulins
Aspirin – prevent clotting in diseased arteries
Other immunosuppressive agents
768
what is the differential diagnosis for a erythemasotus maculopapulous rash + fever
```
Measles
Rubella
Enteroviruses
Cytomegalovirus
Human herpes virus 6/7
Parovirus B19
Epstein- Barre virus
```
769
what is the differential diagnosis for a vesiculobulous rash + fever
Varicella – zoster virus
Herpes simplex virus
Enterovirus
770
what are the different locations of the vesicles in herpes simplex 1, zoster and hand/foot/mouth
simplex - tongue/ mouth
zoster - one dermatome
hand foot mouth
771
what virus cause shingles
herpes zoster
772
how may you differentiate between a vesiculobulbous rash
smear - scrape ulcer base
PCR
immunofluorescence
serology (not acute)
773
is shingles seen in children commonly
no - only immunodeficiency
774
what is the incubation period of varicella zoster virus
14 days (10-21 days)
775
what are complications of varicella zoster virus infections
secondary strep/staph infections skin (10-15%) – cellulitits, strep A leading to toxic shock
meningoencephalitis, cerebellitis,
arthritis
776
what is the treatment for varicella zoster virus infections
aciclovir - normally not needed
777
what percentage of children >5 will have suffered form varicella infection
>90%
| 2-% by 1
778
what babies does fatal varicella occur in (very rare)
1st month of life
| immunodeficiency - T cell deficiency
779
what are complicatiosn of herpes simplex infection
kerato) conjunctivitis
encephalitis
systemic neonatal infections
780
how is congenital herpes caught
direct contact in the birth canal from mother with herpes type 2
781
when and how - does congenital hereps present
day 4-21
70-80% disseminated/CNS infections – Sepsis, Meningoencephalitis, Hepatitis (jaundice, bleeding)
20-30% skin/eye/mouth (SEM) disease
782
what is the prevalence of HSV 1 in children, young adults and adults
20-30% children 5 yrs of age,
40-50% adolescents,
60-80% adults
783
what are the main causes of hand, foot and mouth disease
coxsackie A16
| enterovirus 71
784
what are severe complications of hand, foot and mouth disease
meningitis, encephalitis, acute paralysis, neonatal spies, myocarditis/ pericarditis, hepatitis and chronic infection in immunocompromised patients in neonates
785
what is the difference between a primary and secondary immunodeficiency
primary - body’s immune system is missing or functions improperly
secondary - Components of the immune system itself are all present and functional.
Acquired diseases affecting the immune system and/or treatments negatively influencing the immune system.
786
what mutations cause primary immunodeficiencies
single gene defects
787
what are antibody deficiencies characterised by
deficiency of one of more (sub)classes of antibodies (e.g. IgG, IgA, IgM, IgG2) due to defective B-cell function
Absence of mature B-cells
788
how do antibody deficiencies normally present and by what organism
Presents as recurrent bacterial infections of the upper and/or lower respiratory tract
Mostly caused by S. pneumoniae, H. influenzae
789
what are cellular immunodeficiencies characterised by
impaired T-cell function or the absence of normal T-cells
790
how do cellular immunodeficiencies normally present and by what organism
Presents as Unusual or opportunistic infections often combined with failure to thrive
Pneumocystic jirovecii, CMV (normally asymptomatic infections – not pneumonia)
791
what are innate immune disorders characterised by
defects in phagocyte function
complement deficiency
Absence or polymorphisms in Pathogen Recognition Receptors
792
what organsisms are likely to cause infections in innate immune disorders
```
defects in phagocyte function - S. aureus (sepsis, skin lesions, abscesses internal organs
e.g liver)
Aspergillus infections (lung, bones, brain)
Complement deficiencies - N. meningitidis
```
793
what things may make you suspect a primary immunodeficiency
Severe – requires hospitalisation or IV antibiotics
Persistent – wont completely clear up or clears very slowly
Unusual – caused by an uncommon organism
Recurrent – keeps coming back
Runs in the family – others have had similar susceptibility to infection
794
what are the most common 3 secondary immunodeficiencies
HIV
| chemo/radiotherapy - destroys neutrophils and lymphocytes
795
why is HIV in children very rare
mums tested + treated in 1st trimester
796
how may HIC present in children
```
¥ Recurrent common childhood RTI’s
¥ Persistent oral thrush
¥ Erythematous papular rash
¥ Generalized lymphadenopathy
¥ Recurrent/disseminated VZV/HSV infections (shingles in children)
¥ Failure to thrive
¥ Developmental delay’s
¥ Opportunistic infections: CMV pneumonia/retinitis, PCP (Pneumocystic jiroveci pneumonia)
```
797
how is chronic granulomatous disease inheritd
65% X linked
| 35% ar
798
what is the most common cause of death in chronic granulomatous disease
aspergillosis - severe fungal disease
799
what complex is effected in chronic granulomatous disease
NADPH oxidase complex
800
what test diagnoses chronic granulomatous disease
DHR test - Non-fluorescent rhodamine derivative oxidized by NADPH oxidase to a green fluorescent compound (flow cytometry) – negative test gives diagnosis
801
what is the the curative treatment of chronic granulomatous disease
Stem cell transplant (HSCT)
802
why are fungal infections seen in children in PICU after abdominal surgery/ broad spectrum antibiotics
damage to gut flora favours growth of candida
803
what disease does aspergillus fumigates cause most commonly
pneumonia
804
how may neonatal candidaemia present
2nd/3rd week of life – sepsis syndrome, thrombocytopenia (more than bacterial), hyperglycaemia
attribute to 20-40% mortality
805
what are risk factors for neonatal candidaemia
premature
| low birth weight <750g
806
up to how many self limiting viral infections a year is normal in children
8
807
how may you diagnosis an antibody deficiency
1st serum sample to measure IgG, IgA, IgM
2nd serum sample to measure IgG subclasses (prevent recurrent infections)
3rd serum sample to measure immune response to vaccination with S. pneumoniae and H. influenza (should be protected)
808
how may you diagnosis a complement deficiency
measurement of complement activation and/ or individual factors
809
how are most primary immunodeficiencies treated
prophylactic antibiotics
| extended vaccination schedule
810
do children with tumour lysis syndrome need more fluids or less fluids
more fluids to wash out
811
what should you never give to a patient with an oncological emergency
potassium
812
what % of births have congenital anomalies
3%
813
what re the main causes of multiple congenital anomaly syndromes
¥ single gene disorders 30%
¥ 10% chromosomal
¥ 5% teratogens/ viruses
¥ 55% unknown – mostly
814
what is an 'association' with a syndrome
two features or more features occur together more often than expected by chance
mechanism unclear
815
describe the VATER association
¥ vertebral anomalies (may influence rib anomaly)
Ð ano-rectal atresia
Ð tracheo-oesophageal fistula
Ð radial anomalies
816
what does catch 22 stand for
```
Cleft palate
Abnormal facies – almond shaped eyes, simple ear
Thymic hypoplasia / immune deficiency
Calcium problems
Heart problems
22 - Caused by 22 deletion
```
817
give features of turners syndrome
co-arctation of aorta
short stature
neck webbing
Lymphoedema (Puffy feet, nuchal translucency / cystic hygroma)
Increased carrying angle at elbows, low hairline, wide sp nipples, sandal gap
learning difficulties
primary amenorrhoea and infertility
818
give some features of downs syndrome
¥ Learning disability – key feature, need support to lead safe life
¥ Congenital heart disease
¥ Hypothyroidism – monitored annually
¥ Immunity – increased risk of infections
Early onset Alzheimer disease
819
what is a syndrome
A distinct group of symptoms & signs which, associated together, form a characteristic clinical picture or entity – pattern of recognizable features.
820
how would you investigate the genetic cause of a learning disability
Basic - Microarray, Fragile X
Targetted tests driven by phenotype – clinical judgement
Trio based Exome vs. genome trio analysis (Funding remains an issue)
Captured DNA from exome – next data sequencing – compared with parents
821
what is the difference between a deformation and disruption pattern of development
Deformation: Organ parts are there just not functioning properly
Disruption: Parts of organ / body part absent
822
give an example of a deformation and disruption pattern of development
amniotic bands cutting of bloody supply
823
give 2 examples of sequence malformations
Pierre-Robin sequence - small chin to cleft palate (tongue pushes up as has nowhere to go so the palate doesn’t form properly
Fetal akinesia sequence - reduced fetal movement > reduced breathing > lung hypoplasia
contractures – get stuck
824
what is the difference in features between polydactyly, polysyndactylty and acrocephalopolysyndatyly
extra fingers
+ skin in fingers
+ tall forehead
825
what head features may be seen in a malformation syndrome
¥ Head – shape (brachiocephaly, talucephaly), size (macrocephaly, microcephaly, flat occiput)
Measure occipit- frontal circumference – plot on chart
826
what eye features may be seen in a malformation syndrome
Hypertelorism - Inner canthal distance ICD and inter-pupillary distance IPD
Increased (further apart) – midline brain malformation
Telecanthus / epicanthic folds - ICD increased and IPD normal
827
what ear signs may be seen in a malformation syndrome
ear position (low set, posteriorly rotated (reflects immaturity), small, crumpled
828
what changes in the hands may be seen in a malformation syndrome
```
Finger length, Digital abnormalities (polydactyly extra, oligodactyly less,
Palmar creases (Single palmar crease in Downs)
Marfans- long finger, measure middle finger length/ total hand length >44%
```
829
what is the term used for recognising of a pattern of a syndrome
gestalt
830
what is the standard first line test for malformation syndromes
microarray
831
what is the difference between haematuria and proteinuria
blood in urine
| protein in urine
832
what is the difference in a neonates GFR compared to an adults
20-30ml/min/1.73m
at 2- 80-120
833
what are the 5 main functions of the kidney
1. Waste handling – urea, creatinine
2. Water handling
3. Salt balance – NA, K, PO4, Cl,
4. Acid base control
5. Endocrine - erythropotien, renin, blood pressure, bone health
834
what does proteinuria signify
glomerular injury
835
what proteins make up the glomerular basement membrane
Type IV collagen and laminin
(Synthesis from podocytes and endothelial cells
Mesangial cells playing a role in turnover)
836
what are the functions of the mesangial cells in the kidney
Glomerular structural support
Regulates blood flow of the glomerular capillaries (form biomechanical unit with capillaries capable of regulating filtration surface area as well as intraglomerular blood volume)
837
what things form the glomerular filtration barrier
fenestrated endothelial cell
glomerular basement membrane (GBM)
podocyte with their “slit diaphragms”.
838
which component of the glomerulus is affected in MCD, FSGS, Lupus
epithelial cell (podocyte) injury
839
which component of the glomerulus is affected in Membranous glomerulopathy, MPGN, PIGN
basement membrane
840
which component of the glomerulus is affected in ¥ Infection associated glomerulonephritis (PIGN), Haemolytic Uraemic Syndrome, Membranoproliferative Glomerulonephritis (MPGN), and Lupus
endothelial cell
841
which component of the glomerulus is affected in HSP, IgA vasculitis/ nephropathy and Lupus
mesangial cell
842
which component of the glomerulus is affected in minimal change disease
podocyte
843
which component of the glomerulus is affected in IgA vasculitis
mesangial cell
844
what are the 3 main features of nephrotic syndrome
proteinuria
hypoalbuminaemia
oedema
845
why do you get oedema in nephrotic syndrome
lose protein from urine drops oncotic pressure which drives water into the 3rd space
846
what are the main 3 tests for proteinuria
urine dipstix
protein: creatine urine
24hr urine collection
847
what urine dipstick marker = proteinuria
> 3+
848
how would a Pr:Cr indicate proteinuria
normal: Pr:CR ratio <20mg/mmol
Nephrotic range: >250mg/mmol (10x )
(best early morning, creatine excreted constantly by kidney)
849
how would a 24 hour urine collection indicate proteinuria
normal <60mg/m²/24hrs
| Nephrotic range>1g/m²/24hrs (Adults >3.5g/24hrs)
850
what is urine Na a test of
tells if intravascular space dry
| body conserve water for intravascular volume by retaining sodium (low urine sodium
851
what is the most common nephrotic syndrome in children
minimal change disease
| 80-90% steroid sensitive
852
how is the diagnosis of minimal change disease made
trial of steroids
853
what is the treatment of minimal change disease
8 weeks prednisolone
854
what are typical features of minimal change disease
5 years, normal blood pressure, haematuria (microscopic) but normal renal function.
855
what are atypical features of minimal change disease that will require a biopsy
autoimmune conditions
| steroid resistance
856
what are some SE of long term steroids
more susceptible to infections, glucocorticoid toxicity from treatment, GI side effects, glucose intolerance, hypertension, risk of adrenal crisis, altered growth, behaviour (irritable, moody, don’t sleep) Check varicella status/ pneumococcal vaccination
857
what is the outcome of treatment with prednisolone for minimal change disease
95% in 2-4 weeks,
| 80% relapse, 50% frequent
858
what drugs would a child be put on if the have >4 relapses of MCD / year
```
immunosuppression -
Levimazole,
Cyclosporin,
Tacrolimus, ,
Rituximab (CD20 monoclonal antibody)
Alkylating agent (cyclophosphamide oral / nitrogen mustard IV),
```
859
what is the risk of focal segmental glomerulosclerosis
end stage renal failure - need immunosuppression
860
what congenital mutations will cause an <3 month infant to present with nephrotic syndrome
NPHS1 – nephrin/ NPHS 2 – podocin
861
when would you investigate microscopic haematuria
Investigate > trace on 2 occasions or gross haematuria (persistent)
862
name some things that may cause macroscopic haematuria
```
UTI (dysuria) – most common cause
Trauma
Stones (pain)
Glomerulonephritis – Post infective GN, IGA/ HSP, Membranoproliferative GN, lupus nephritis
Benign recurrent haematuria (FH)
Alports syndrome
HUS
Tumour (WIlm’s)
Clotting abnormalities
```
863
what class of AKI is caused by nephritic syndrome
intrarenal
864
how would you investigate haematuria
Creatinine, FBC, Albumin, Urine
Exclude UTI – urine culture negative
Radiology (check anatomy), ANA/ ANCA antibodies (exclude auto-immune disease), biopsy
865
what is a feature of fluid overload seen in the neck
raised JVP
866
what bacteria usually causes post infectious GN
group A strep- b haemolytic
| 5-21 days after infection, throat (7-10 days) or skin (2-4 weeks)
867
what is the pathogenisis of post infectious GN
antigen-antibody complex deposition in kidney – activates complement (levels drop)
868
what is the prognosis of post infectious GN
most clear within 6 months and don't reoccur
869
how would you diagnose post infectious GN
bacterial culture, positive ASOT, low C3
870
what is the treatment of post infectious GN
antibiotics (10 days penicillin), support renal function, volume overload - diuretics
871
what is the most common cause of GN in adults worldwide
IgA nephropathy
872
how many days after a upper respiratory tract infection would IgA nephropathy present
1-2 days
873
what are clinical signs in the urine of IgA nephropathy
Recurrent macroscopic haematuria (frank)
chronic microscopic haematuria
Varying degree of proteinuria
874
how is IgA nephropahty investigated
Clinical picture + Biopsy
| Normal complement, negative autoimmine
875
how is IgA nephropahty treated
treat proteinuria – ACEi,
| moderate disease – immunosuppression
876
what is henoch schonlein purpura
IgA related vasculitis
877
when is the onset of henoch schonlein purpura
5-15 years
878
what are diagnostic features of henoch schonlein purpura
Mandatory palpable purpura + one of 4
1. Abdominal pain
2. Renal involvement
3. Arthritis or arthralgia (joints)
4. Biopsy - IgA deposition + complement
879
give causes of nephritic syndrome
```
post infectious GN
IgA nephropathy
Henoch Schonlein purpura
membranoproliferative GN
lupus nephritis
ANCA positive vasculitis
```
880
what is the trigger for IgA vasculitis in most children
viral URTI - strep
881
how long does the rash last in IgA vasculitis
4-6 weeks
882
what cell in injured in IgA vasculitis
mesengial
883
what is the treatment of IgA vascultisi
symptomatic - joints, gut
Glucocorticoid therapy - (GI involvement)
Immunosuppression – prednisolone, cyclophosphamide
884
what is an AKI
abrupt loss of kidney function, resulting in the retention of urea and other nitrogenous waste products and in the dysregulation of extracellular volume and electrolytes
885
what are signs of AKI
¥ Anuria/oliguria (<0.5ml/kg/hr) > 8 hours
¥ Rapid rise in plasma creatinine
¥ Hypertension with fluid overload
¥ Serum creatinine: > 1.5x age specific reference creatinine
886
how much does the serum creatinine rise in AKI
>1.5x
887
what is the difference in the creatine levels in AKI 1-3
1 - >1.5x baseline
2 - 2-3x baseline
3- >3x
888
what are some pre-renal causes of AKI
```
Volume depletion hypoperfusion of kidneys
Oedematous states
Hypotension
hypoperfusion – Drugs
renal artery stenosis or occlusion
Hepato-renal syndrome.
```
889
what are some post -renal causes of AKI
obstructive nephropathy
890
what are some intrinsic causes of AKI
Glomerular disease – HUS, Glomerulonephritis
Tubular injury - acute tubular necrosis (ATN), Consequence of hypoperfusion, Drugs
Interstitial nephritis - NSAID, autoimmune
891
what is the management of AKI (3M's)
prevention
Monitor – Urine Output, PEWs, BP, weight (water)
Maintain – good hydration
Minimise – drugs (ibuprofen)
892
what is monitored long term after an AKI
blood pressure and proteinuria - risk of CKD
893
what are the treatment principles of treating metabolic bone disease
low phosphate diet
phosphate binders (Ca)
Active vit D to control PTH
894
what is the role of PTH and how is this affected in end stage renal disease
PTH balances Ca in bones
PTH helps kidneys to excrete phosphate
if kidneys can't excrete phosphate then PTH increases and Ca drops
895
what is the best way to measure blood pressure in under 5s
doppler form wirst
896
what is hypertension defined as in young children
>95th percentile
| borderline >90 but <95th – affected by age, sex, height, weight
897
give factors that affect the progression of CKD
```
Late referral
Hypertension
Proteinuria
High intake of protein, phosphate and salt (CKD – normal protein, low phosphate)
Bone health - Vitamin D, PTH, Phosphate
Acidosis
Recurrent UTIs
```
898
what are the grades of vesico- uteric reflux
1-ureter only
2-ureter, pelvis, calyces (no distension)
3-dilatation ureter
4-Moderate dilatation of ureter ± pelvis ±tortuous ureter, obliteration of fornices
5-gross dilatation/tortuosity, no papillary impression in calyces
899
what are the guidelines for investigating UTI in children
Upper tract symptoms (fever, vomiting, systemically unwell), Younger (<6 months high risk), Recurrent
900
what imaging is done to look at the anatomy of the kidneys
ultrasound
901
what scan is done to look for scarring of the kidneys
DMSA (isotope scan)
902
what scans are done to look for urine reflux
Micturating cystourethrogram/ MAG 3 scan
903
what is the treatment of a lower UTI
3 days oral antibiotics -
| - Trimethoprim, Co-amoxiclav, cephalosporin
904
how would you treat an upper tract UTI or pyelonephritis
antibiotics for 7-10 days
oral if well - Trimethoprim, Co-amoxiclav, cephalosporin
IV - 3rd generation Cephalosporin or Co-amoxiclav
905
what are complications of UTI
scarring > hypertension
| AKI
906
how may you diagnose a UTI
Dipstix – WCC, Leucocyte esterase activity, nitrites (+VE = UTI)
Microscopy - Pyuria >10 WBC per cubic mm
Bacturia
Culture > 10^5 Colony forming units /ml - (gold standard)
907
what is the most common cause of UTI in kids
E.coli
| Klebsiella, Proteus (stones), Strep Faecalus
908
what is needed to diagnose a UTI
clinical signs
+ Bacteria culture from midstream urine
Any growth on suprapubic aspiration or catheter
909
what are signs of an upper urinary tract infection
fever, vomiting, lethargy, irritability, abdominal pain/ tenderness, poor feeding
910
what methods can be used to obtain a urine sample in children
- Clean catch urine or midstream urine in adults/ older
- collection pads, urine bags
- catheter samples or suprapubic aspiration (USS)
911
what hereditary conditions can cause CKD in children
Cystic kidney disease
Cystinosis
Syndromic - Turner, Trisomy 21, Branchio-oto-renal, Prune Belly syndrome
bilateral cryptorchidism
912
what does the GFR drop to before symptoms of CKD are seen
<60
913
what is the most common cause of haemolytic uraemia syndrome
post diarrhoea
| Entero-Haemorrhagic E.coli (EHEC)
914
what organism commonly causes haemolytic uraemia syndrome
e.coli 0157
915
after onset of bloody diarrhoea, how long is HUS a risk for?
14 days
916
what is the triad of HUS
Microangiopathic haemolytic anaemias
Thrombocytopenia
Acute renal failure
917
how is HUS treated
3Ms:
Monitor: Fluid balance, electrolytes, acidosis
Hypertension
Aware of other organs - Seizures, acute abdomen, diabetes, adrenal crisis
Maintain: IV normal saline and fluid – reduce oliguric HUS by fluid expansion + salt
Renal replacement therapy – 30-40% need RRT
Minimise: No antibiotics
918
how does alport syndrome present
haematuria, proteinuria, hypertensions, early renal failure (20-30)
deafness
eye changes - lenticonus, macular changes in retina
919
why do people with aport syndrome get deafness
collagen in cochlear membrane - SN deafness
920
why do people with aport syndrome get lenticonus
collagen in decements membrane
921
what are some acquired causes of cystic renal diabetes
cancer
Autosomal dominant glomerulocystic kidney disease
Early onset diabetes mellitus (MODY)
Genetic heterogeneity - HNF1β mutations
922
what is seen in developmental cystic renal disease
Dysplasia/ Multicystic dysplastic (MCDK)
Usually sporadic, Non-functioning kidney, usually unilateral.
Ureteric atresia + Hypertrophy of the normal contralateral kidney
923
what are some congenital causes of cystic renal disease
Autosomal Recessive (ARPKD)
Autosomal Dominant (ADPKD)
Juvenile Familial Nephronophthisis (JFN) - normal BP
Potters sequence
924
what are extra renal features of Juvenile Familial Nephronophthisis (JFN)
```
normal BP
Syndromic:
Retinitis pigmentosa;
Hepatic fibrosis;
Skeletal dysplasia;
CNS abnormalities.
```
925
what are extra renal features of ARPKD
severe hypertension
Hepatic fibrosis →
Portal hypertension.
Bacterial cholangitis.
926
what are extra renal features of ADPKD
severe hypertension
Extrarenal cysts (liver and/or pancreas)
Cerebral aneurysms in 8% - familial clustering
Paediatrics
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