paeds Flashcards
(754 cards)
1
Q
what are the three fetal shunts
A
ductus venosus, foramen ovale, and ductus arteriosus
2
Q
ductus venousus shunts
A
from the umbilical vein to the IVC to bypass the liver
3
Q
foramen ovale shunts
A
right atrium to left atrium to bypass right ventricle and pulmonary circulation
4
Q
ductus arteriosus shunts
A
the pulmonary artery with the aorta to bypass pulmonary circulation
5
Q
why does the foramen ovale close?
A
first breath expands alveoli decreasing pulmonary vascular resistance causes pressure to fall in the right atrium which squashes the atrial septum.
6
Q
foramen ovale after shutting gradually becomes the
A
fossa ovalis
7
Q
what is required to keep the ductus arteriosus open?
A
prostaglandins
8
Q
why does the ductus arteriosus close after first breath
A
increased blood oxygen decreases circulating prostaglandins
9
Q
after closing the ductus arteriosus forms
A
ligamentum arteriosum
10
Q
ductus venosus closes because
A
of umbilical cord clamping
11
Q
ductus venosus becomes
A
ligamentum venosum
12
Q
innocent murmurs are
A
systolic flow murmurs
13
Q
features of an innocent murmur are
A
soft, short, systolic, asymptomatic, and situation dependent.
14
Q
red flags for a murmur are
A
loud, diastolic, louder on standing or systemic features.
15
Q
investigations for murmurs are
A
ECG, CXR and echo
16
Q
pan systolic murmur examples are
A
mitral regurgitation, tricuspid regurgitation, and ventricular septal defects
17
Q
ventricular septal defects are heard at the
A
lower left sternal border
18
Q
ejection systolic murmurs examples
A
aortic stenosis, pulmonary stenosis and hypertrophic obstructive cardiomyopathy
19
Q
hypertrophic obstructive cardiomyopathy is heard at
A
fourth intercostal space on the left sternal border
20
Q
negative thoracic breathing during inspiration causes
A
physiological splitting of the heart sound
21
Q
atrial septal defect murmur
A
mid systolic crescendo decrecscendo murmur with a fixed split second heart sound
22
Q
patent ductus arteriosus murmur
A
continous crescendo-decrescendo machinery murmur
23
Q
tetralogy of fallot murmur
A
ejection systolic murmur due to pulmonary stenosis
24
Q
cyanotic heart disease pathology
A
deoxygenated blood in systemic circulation. occurs with a right to left shunt.
25
heart defects that cause right to left shunt (cyanotic heart disease)
VSD, ASD, PDA, and transposition of the great arteries
26
risk factors for patent ductus arteriosus (PDA)
rubella or prematurity
27
left to right shunt increases pressure in the
pulmonary hypertension and causes right heart strain due to increases resistance leading to hypertrophy.
28
presentation of PDA
SOB, difficulty feeding, poor weight gain, lower respiratory tract infection
29
management of PDA
monitor with ECHO until 1 as may close conservatively.
30
surgical management of PDA
trans-catheter or surgical closure.
31
the separation of the upper chamber of the heart (the atria) during development is by the
two walls that form with the endocardial cushion
32
the name of the two walls that separate the atria are
septum primum and septum secondum
33
pulmonary hypertension may lead to what syndrome
Eisenmenger syndrome
34
most common atrial septal defect is
ostium secondum
35
types of atrial septal defect are
ostium secondum, patent foramen ovale, ostium primum
36
27 year old male develops a DVT after post operation that causes a massive stroke but as to the cause, no one is sure. what may you suspect?
lifelong asymptomatic ASD
37
complications of ASD?
stroke, atrial fibrillation, pulmonary hypertension with right sided heart failure, and eisenmenger syndrome.
38
presention of ASD in adulthood are
dyspnoea, heart failure or stroke
39
ASD presentation in childhood
asymptomatic, Shortness of breath
Difficulty feeding
Poor weight gain
Lower respiratory tract infections
40
management of ASD is
conservative, transverse catheter closeure via femoral vein, open heart surgery or anticoagulants
41
ventricular septal defects genetic associations
down's syndrome and turner's syndrome
42
symptoms of VSD are
```
initially asymptomatic but Poor feeding
Dyspnoea
Tachypnoea
Poor feeding
Failure to thrive
```
43
VSD may be corrected by
transvenous catheter closure via femoral vein or open heart surgery
44
risk of what infection is likely with a VSD?
infective endocarditis,
45
bone marrow response to cyanosis in eisenmenger's syndrome
production of more RBS leading to polycythaemia causing a plethoric complexion.
46
complication of polycythaemia?
increases likelihood of clots.
47
pulmonary hypertension examination signs
raised JVP, right ventricular heave, loud P2, peripheral oedema
48
right to left shunt and chronic hypoxia findings
Cyanosis
Clubbing
Dyspnoea
Plethoric complexion
49
mortality prognosis in eisenmenger syndrome
reduced life expectancy of 20 years
50
can you medically reverse eisenmenger syndrome?
no
51
treatment of eisenmenger syndrome could be through a
heart and lung transplant
52
eisenmenger syndrome pulmonary hypertension mx
sildenafil
53
eisenmenger syndrome mx of polycythaemia
venesection
54
eisenmenger syndrome mx of infective endocarditis
prophylactic antibiotics
55
aortic coarctation is related to
turner's syndrome
56
only indication of aortic coarctation may be
weak femoral pulses
57
investigation of coarctation may be through
four limb blood pressure, high blood pressure will arise from the limbs supplied before the narrowing
58
signs of coarctation
systolic murmur below left clavicle, tachypnoea, poor feeding and grey/floppy baby
59
later in life signs of coarctation
left ventricular heave, underdeveloped limbs
60
management of coarctation
critical care require prostaglandin with surgery to ligate the ductus arteriosus and correct the coarctation.
61
the aortic valve is called
the three leaflets are called the aortic sinuses of valsalva
62
significant aortic stenosis is causes
fatigue, shortness of breath, dizziness and fainting. worse on exertion and likely to present with heart failure.
63
aortic stenosis ejection systolic murmur may radiate to the
carotids
64
examination signs of aortic stenosis also includes
ejection click before the murmur, palpable thrill and slow rising pulse with narrow pulse pressure.
65
gold standard investigation for aortic stenosis is
ECHO
66
additional Ix for aortic stenosis is
ECGs and exercise testing
67
treatment for aortic stenosis includes
Percutaneous balloon aortic valvoplasty
Surgical aortic valvotomy
Valve replacement
68
complication of aortic stenosis is
```
Left ventricular outflow tract obstruction
Heart failure
Ventricular arrhythmia
Bacterial endocarditis
Sudden death
```
69
pulmonary valve consists of how many leaflets?
three
70
pulmonary valve stenosis is associated with
Tetralogy of Fallot
William syndrome
Noonan syndrome
Congenital rubella syndrome
71
presentation of pulmonary valve stenosis
symptoms of fatigue on exertion, shortness of breath, dizziness and fainting.
72
signs of pulmonary stenosis includes
ejection systolic murmur, palpable thrill, right ventricular heave and raised JVP with giant a waves.
73
symptomatic pulmonary stenosis treatment is through
balloon valvuloplasty via venous catheter via femoral vein or open heart surgery.
74
tetralogy of fallot consists of
Ventricular septal defect (VSD)
Overriding aorta
Pulmonary valve stenosis
Right ventricular hypertrophy
75
risk factors for tetralogy of fallot
Rubella infection
Increased age of the mother (over 40 years)
Alcohol consumption in pregnancy
Diabetic mother
76
CXR may show tetralogy of fallot as
a boot shaped heart
77
severe cases of tetralogy of fallot will cause what before one year?
heart failure
78
signs and symptoms of tetralogy of fallot
```
Cyanosis
Clubbing
Poor feeding
Poor weight gain
Ejection systolic murmur
“Tet spells”
```
79
tet spells refer too
temporary worsening of right to left shunt causing a cyanotic episodes
80
tet spells pathology are
pulmonary vascular resistance increasing or systemic resistance decreasing due to presence of carbon dioxide
81
why does carbon dioxide causes a decrease in systemic vascular resistance?
vasodilator.
82
tet episodes may be triggered by?
waking, physical exertion or crying
83
tet spells initial management
squatting to increase systemic pressure
84
medical management of tet spell
oxygen, beta blockers, IV fluids, morphine, sodium carbonate and phenylephrine infusion.
85
management of tetralogy of fallot
prostaglandin infusion to maintain ductus arteriosus, total surgical repair or open heart surgery.
86
ebsteins anomaly refers too
lower tricuspid valve in right side of heart.
87
anatomically ebstein's anomaly causes
large right atrium but small right ventricle
88
ebsteins anomaly is related to what syndrome
wolff-parkinson-white syndrome
89
presentation of ebstein's anomaly is
```
Evidence of heart failure (e.g. oedema)
Gallop rhythm
Cyanosis
Shortness of breath and tachypnoea
Poor feeding
Collapse or cardiac arrest
```
90
ECG findings for ebsteins anomaly includes
Arrhythmias
Right atrial enlargement
Right bundle branch block
Left axis deviation
91
medical management of ebsteins anomaly is for
treating arrhythmias and heart failure
92
transposition of the great vessels anatomically means?
RV pumps into aorta and LV pumps into pulmonary vessels
93
immediate survival of the baby with transposition of great vessels is through
patent ductus arteriosus, atrial septal defect or ventricular septal defect.
94
transposition of the great vessels is usually identified through
antenatal ultrasound scans
95
how may a balloon septostomy assist in transposition of great vessels.
via umbilicus a catheter can open a atrial septal defect
96
definitive management of transposition of great vessels is
open heart surgery
97
type 1 diabetes may be triggered by what viruses
Coxsackie B virus and enterovirus.
98
functions of insulin are
enables glucose entry to the cell, and enable muscle, livers cells to produce glycogen.
99
insulin is an example of what sort of hormone
anabolic
100
insulin is produced by
pancreas, beta cells in the islets of langerhan
101
glucagon is an example of what sort of hormone
catabolic
102
glucagon is produced by
alpha cells in the islets of langerhan in the pancreas.
103
glucagon function is
liver to breakdown glycogen via glycogenolysis, and for the liver to convert fats into glucose through gluconeogenesis
104
ketogenesis occurs through
liver using fatty acids to create water soluble fatty acids
105
people in ketosis have what smell in their breath?
acetone.
106
triad of hyperglycaemia is
polyuria, polydispia and weight loss.
107
other symptoms of hyperglycaemia are
secondary enuresis and recurrent infections.
108
diagnosis of type 1 diabetes should involve
FBC, renal profile and formal lab glucose, and HbA1c.
109
additional tests to consider with a type 1 diabetic
blood cultures for infection, thyroid function tests (autoimmune thyroid disease), coeliac disease and insulin antibodies.
110
insulin injections in the same spot may cause
lipodystrophy
111
patients with type 1 diabetes usually started on
basal bolus regime
112
example of basal is
long acting insulin lantus usually given in the evening
113
example of bolus is
short acting insulin actrapid, usually three times a day according to carb intake.
114
two types of insulin pump are
tethered or patch
115
hypoglycaemia symptoms include
unger, tremor, sweating, irritability, dizziness and pallor. More severe hypoglycaemia will lead to reduced consciousness, coma and death
116
first line for hypoglycaemia is
lucozade
117
severe hypoglycaemia consider
IV dextrose and intramuscular glucagon.
118
differentials for a hypoglycaemia episode
hypothyroidism, glycogen storage disorders, growth hormone deficiency, liver cirrhosis, alcohol and fatty acid oxidation defects
119
common complication of type 1 diabetes in kids are
nocturnal hypoglycaemia
120
triad of long term complications of chronic hyperglycaemia
macrovascular, microvascular and infection
121
macrovascular complications of hyperglycaemia
coronary artery disease, diabetic foot, stroke, hypertension.
122
microvascular complications of hyperglycaemia
peripheral neuropathy, retinopathy, kidney disease (glomerulosclerosis)
123
monitoring of blood sugars can be through
Hb1Ac every 3-6 months, capillary glucose or FreeStyle libre.
124
triad of outcome of diabetic ketoacidosis
ketoacidosis, dehydration and potassium imbalance
125
pathology of acidosis in DKA
ketones increase acid levels, bicarbonate before but eventually run out
126
pathology of dehydration in DKA
glucose leaks into urine, osmotic diuresis draws water into the urine causing polyuria and results in dehydration
127
potassium imbalance pathology in DKA
insulin normally drives potassium into cells. leads to arrhythmias.
128
priority of treatment in DKA is
fluid resus followed by insulin infusion.
129
DKA complication of treatment is
cerebral oedema
130
pathology of cerebral oedema via DKA tx
fluid moves to extracellular space in DKA. fluids and insulin cause rapid shift of fluid into intracellular space causing cells to swell.
131
signs of cerebral oedema
headaches, altered behaviour, bradycardia or changes to consciousness.
132
management of cerebral oedema in DKA tx
slowing IV fluids, IV mannitol and IV hypertonic saline.
133
symptoms of DKA
```
Polyuria
Polydipsia
Nausea and vomiting
Weight loss
Acetone smell to their breath
Dehydration and subsequent hypotension
Altered consciousness
sepsis
```
134
diagnosis of DKA is
Hyperglycaemia (i.e. blood glucose > 11 mmol/l)
Ketosis (i.e. blood ketones > 3 mmol/l)
Acidosis (i.e. pH < 7.3)
135
rehydration of DKA should occur over
evenly over 48 hours
136
principles of treatment for DKA
avoid bolus, treat underlying triggers, avoid hypoglycaemia, add potassium to fluids, monitor for cerebral oedema.
137
adrenal insufficiency refers too when
adrenal glands do not produce enough steroids particularly cortisol and aldosterone.
138
addison's disease refers too
primary adrenal insufficiency of cortisol and aldosterone
139
common cause of addison's disease is
autoimmune
140
secondary adrenal insufficiency refers too
inadequate ACTH
141
causes of secondary adrenal insufficiency includes
damage to pituitary, congenital, infection, surgery, RT or loss of blood.
142
tertiary adrenal insufficiency refers too
inadequate CRH release by the hypothalamus
143
cause of tertiary adrenal insufficiency is
sudden withdrawal or exogenous long term steroids (>3 weeks)
144
features of adrenal insufficiency in babies
```
Lethargy
Vomiting
Poor feeding
Hypoglycaemia
Jaundice
Failure to thrive
```
145
features of adrenal insufficiency in children
```
Nausea and vomiting
Poor weight gain or weight loss
Reduced appetite (anorexia)
Abdominal pain
Muscle weakness or cramps
Developmental delay or poor academic performance
Bronze hyperpigmentation
```
146
investigations for adrenal insufficiency
U&Es (hyponatraemia and hyperkalaemia) and blood glucose (hypoglycaemia) as well as cortisol, ACTH, aldosterone and renin levels
147
addison's disease specific hormone levels
Low cortisol
High ACTH
Low aldosterone
High renin
148
secondary adrenal insufficiency hormone levels.
Low cortisol
Low ACTH
Normal aldosterone
Normal renin
149
what specific test can be performed to confirm adrenal insufficiency
short synacthen test
150
short synacthen testing involves
baseline cortisol measure, injection then measure 30 and 60 minutes post.
151
short synacthen test is a failure when
cortisol fails to rise less than double the baseline confirming addison's disease.
152
glucocorticoid replacement for adrenal insufficiency is
hydrocortisone (cortisol)
153
mineralcorticoid hormone replacement for adrenal insufficiency is
fludrocortisone (aldosterone)
154
daily monitoring post adrenal insufficiency involves
```
Growth and development
Blood pressure
U&Es
Glucose
Bone profile
Vitamin D
```
155
sick day rules for adrenal insufficiency involves
dose of steroid increased and more regularly, blood glucose monitoring, vomiting and diarrhoea involves IM injection of steroid likely.
156
Addisonian crisis presentation
Reduced consciousness
Hypotension
Hypoglycaemia, hyponatraemia and hyperkalaemia
157
management of addisonian crisis involves
I.V. hydrocortisone, intense monitoring of electrolytes, fluid and blood sugars with I.V. resus.
158
congenital adrenal hyperplasia is the deficiency in the
21-hydroxylase enzyme
159
congenital adrenal hyperplasia effects hormones how?
nderproduction of cortisol and aldosterone and overproduction of androgens
160
congenital adrenal hyperplasia genetic pattern is
autosomal recessive
161
mineralcorticoid hormone is released in response to what and for what function?
renin, to control the balance of salt and water in the blood
162
aldosterone acts on the kidney to
increase sodium reabsorption into the blood and increase potassium secretion into the urine. Therefore, aldosterone acts to increase sodium and decrease potassium in the blood.
163
21-hydroxylase is the enzyme responsible for
converting progesterone into aldosterone and cortisol.
164
growth hormone stimulates the release of
insulin-like growth factor 1 (IGF-1) by the liver,
165
empty sella syndrome refers too
under developed pituitary gland.
166
neonate presentation of growth hormone deficiency
Micropenis (in males)
Hypoglycaemia
Severe jaundice
167
older infants present with what? for GH deficiency
Poor growth, usually stopping or severely slowing from age 2-3
Short stature
Slow development of movement and strength
Delayed puberty
168
growth hormone stimulation test involves measuring the response to
glucagon as well as insulin, arginine and clonidine.
169
other investigations for growth hormone deficiency
thyroid and adrenal insufficiency, MRI brain, genetic testing, x-ray or DEXa
170
associated genetic conditions for GH deficiency include
Turner syndrome and Prader–Willi syndrome
171
Tx for GH deficiency is
Daily subcutaneous injections of growth hormone (somatropin)
172
two causes of congenital hypothyroidism are
dysgensis (underdeveloped gland thyroid gland) or dyshormonogenesis.
173
screening for congenital hypothyroidism is through
newborn blood spot screening test.
174
symptoms of congenital hypothyroidism include
```
Prolonged neonatal jaundice
Poor feeding
Constipation
Increased sleeping
Reduced activity
Slow growth and development
```
175
commonest cause of acquired hypothyroidism is
autoimmune thyroiditis known as hashimoto's thyroiditis
176
autoimmune thyroiditis/ hashimoto's thyroiditis pathology
antithyroid peroxidase (anti-TPO) and antithyroglobulin antibodies .
177
hashimoto's thyroiditis is associated with
type 1 diabetes and coeliac disease.
178
symptoms of hashimoto's thyroiditis are
```
Fatigue and low energy
Poor growth
Weight gain
Poor school performance
Constipation
Dry skin and hair loss
```
179
follow up investigations of hashimoto's thyroiditis include
thyroid function blood tests (TSH, T3 and T4), thyroid ultrasound and thyroid antibodies.
180
treatment for hashimoto's thyroiditis
levothyroxine.
181
commonest cause of bronchiolitis
respiratory syncytial virus RSV
182
bronchiolitis commonly occurs in children aged
under 1 year
183
why does RSV not affect adults in the same way as young kids?
proportional size of the airways
184
presentation of bronchiolitis
coryzal symptoms, dyspnoea, tachypnoea, poor feeding, mild fever, apnoeas, wheeze and crackles.
185
signs of bronchiolitis
raised respiratory rate, accessory muscles (SCM, abdominal), intercostal and subcostal recessions, nasal flaring, head bobbing, tracheal tugging, cyanosis and abnormal airway nosies.
186
wheezing is causes by
narrowed airways usually on expiration
187
grunting is caused by
exhaling with a partially closed glottis to increase end expiratory pressure.
188
stridor is caused by
obstruction of upper airway
189
RSV typical course
chesty symptoms day 1-2.
onset of coryzal symptoms with peak being day 3/4.
recovery over week 2 - 3.
190
reasons to admit for bronchiolitis
under 3 months old, prexisitng condition, down's syndrome, prematurity, respiratory rate >70, oxygen sats <92%, severe respiratory distress.
191
management of bronchiolitis
adequate intake via NG tube or IV fluids, saline nasal drops, supplementary oxygen.
192
ventilatory support for bronchiolitis includes
high flow humidified oxygen, continuous positive airway pressure, intubation and ventilation
193
why is high flow humidified oxygen helpful outside oxygenation?
provides end expiratory pressure.
194
how can you assess ventilation?
rising pCO2, falling pH and respiratory acidosis all signs of poor ventilation.
195
prevention of bronchiolitis may be managed through?
Palivizumab a monoclonal antibody given with monthly injections. passive protection.
196
who is in the high risk group for RSV?
congenital heart disease or premature babies.
197
viruses that commonly cause a viral induced wheeze?
RSV or rhinovirus
198
Poiseuille's law dictates
hat flow rate is proportional to the radius of the tube to the power of four.
199
features that differentiate a viral induced wheeze to asthma are
before 3 years old, not history of atopy and only occurs during infection.
200
presentation of viral wheeze is
Shortness of breath
Signs of respiratory distress
Expiratory wheeze throughout the chest
201
focal wheeze indicates
focal airway obstruction
202
acute asthma presentation
Progressively worsening shortness of breath
Signs of respiratory distress
Fast respiratory rate (tachypnoea)
Expiratory wheeze on auscultation heard throughout the chest
The chest can sound “tight” on auscultation, with reduced air entry
203
moderate acute asthma signs
peak flow >50% and normal speech
204
severe acute asthma signs
peak flow <50%, sats <92%, unable to complete sentences in one breath, respiratory distress, hear rate (>140 in 1-5yrs, >125>5yrs), RR (>40 1-5yrs, >30 in >5yrs)
205
life threatening asthma signs
peak flow <33%, sats <92%, exhaustion and poor respiratory effort, hypotension, silent chest, cyanosis, altered consciousness.
206
staples of management of virally induced wheeze or acute asthma are
supplementary oxygen, bronchodilators, steroids and antibiotics.
207
bronchodilators for acute asthma are
salbutamol (Beta 2 agonist), ipratropium bromide (anti-muscarinic), IV magnesium sulphate, IV aminophylline
208
moderate to severe asthma stepwise progression
1. salbutamol
2. nebuliser salbutamol/ipratropium bromide
3. oral prednisolone
4. IV hydrocortisone
5. IV magnesium sulphate
6. IV salbutamol
7. IV aminophylline
209
salbutomal dosage for moderate to severe asthma
10 puffs every 2 hours
210
oral prednisone loading dose
1mg per kg of body weight once a day for 3 days.
211
high doses of salbutamol should make you consider the levels of
potassium as they absorbed into cells
212
post acute asthma steps to consider
Finish the course of steroids if these were started (typically 3 days total)
Provide safety-net information about when to return to hospital or seek help
Provide an individualised written asthma action plan
213
asthma is a
chronic inflammatory airway disease leading to variable airway obstruction.
214
asthma is one atopic condition, what are other examples, perhaps in the family history?
allergies, eczema, asthma, hay fever
215
presentation of asthma
Episodic symptoms intermittent exacerbations
Diurnal variability, t
Dry cough with wheeze and shortness of breath
Typical triggers
Bilateral widespread “polyphonic” wheeze
Symptoms improve with bronchodilator
216
investigations for asthma may include
spirometry, direct bronchial challenge with histamine or methacholine, fractional exhaled nitric oxide, peak flow variability
217
chronic asthma therapy <5yrs
1. SABA
2. Add a low dose corticosteroid inhaler or a leukotriene antagonist (i.e. oral montelukast)
3. Add the other option from step 2.
4. Refer to a specialist.
218
medical therapy for chronic asthma 5-12 years
1. Start a SABA as req.
2. low dose corticosteroid inhaler
3. LABA
4. corticosteroid inhaler to a medium dose.
5. Consider adding:
Oral leukotriene receptor antagonist (e.g. montelukast)
Oral theophylline
6. corticosteroid to a high dose.
7. Referral to a specialist.
219
therapy for chronic asthma >12 years
1. SABA
2. low dose corticosteroid
3. LABA
4. increase corticosteroid dose, trial LAMA, monteleukast, theophylline etc.
5. increase corticosteroid dose.
6. oral steroids.
220
does inhaled corticosteroids effect growth?
effect dose dependent hence the need for good asthma control and regular reviews
221
MDI spacer cleaning tips
once a month, avoid scrubbing and air dry.
222
pneumonia on a cxr presents with
consolidation
223
presentation of pneumonia is
```
Cough (typically wet and productive)
High fever (> 38.5ºC)
Tachypnoea
Tachycardia
Increased work of breathing
Lethargy
Delirium
```
224
general signs of pneumonia include
```
Tachypnoea (raised respiratory rate)
Tachycardia (raised heart rate)
Hypoxia (low oxygen)
Hypotension (shock)
Fever
Confusion
```
225
chest signs of pneumonia include
bronchial breathing, focal coarse crackles, dullness to percussion
226
most common cause of bacterial pneumonia
streptococcus pneumonia
227
staphylococcus aureus signs on CXr with pneumonia
pneumatocoeles (round air filled cavities) and consolidations in multiple lobes.
228
what bacteria may effect unvaccinated or pre vaccinated children with pneumonia
group B strep and haemophilus influenza
229
mycoplasma pneumonia presentation of pneumonia
extra-pulmonary manifestations (e.g. erythema multiforme).
230
commonest viral cause of pneumonia include
RSV the most common
231
other viruses that may cause pneumonia
parainfluenza, and influenza
232
ix for pneumonia
CXR, sputum cultures, throat swab,viral PCR
233
Tx for pneumonia first line antibiotic is
amoxicillin
234
what may be used to treat atypical pneumonia or as alternative to penicillin?
macrolides
235
examples of macrolides include
erythromycin, clarithromycin or azithromycin
236
additional tests in recurrent respiratory tract infection
sweat test, serum immunoglobulins, IgG, sweat test and HIV, FBC and CXR
237
croup commonly effects what age group?
6 months to 2 years
238
croup causes
barking cough due to oedema in the larynx
239
classic cause of croup is
parainfluenza virus
240
croup used to be caused by
diptheria
241
diptheria croup can lead to
epiglottitis
242
presentation of croup
```
Increased work of breathing
“Barking” cough, occurring in clusters of coughing episodes
Hoarse voice
Stridor
Low grade fever
```
243
what treatment is very effective for croup
single dose 150 mcg/kg of oral dexamethasone.
244
severe croup may require.
Oxygen
Nebulised budesonide
Nebulised adrenalin
Intubation and ventilation
245
epiglottitis is typically caused by
haemophilus influenza type B.
246
presentation of epiglottis
```
Patient presenting with a sore throat and stridor
Drooling
Tripod position, sat forward with a hand on each knee
High fever
Difficulty or painful swallowing
Muffled voice
Scared and quiet child
Septic and unwell appearance
```
247
lateral X-ray of epiglottis reveals
thumbprint sign
248
treatment of epiglottitis is
securing the airway (intubation, tracheostomy, ITU) with IV antibiotics (ceftriaxone) and steroids (dexamethasone)
249
common complication of epiglottitis is
abscess
250
laryngomalacia is a condition that refers too
the supraglottis larynx flops
| causing partial airway obstruction
251
in laryngomalcia the aryepiglottic folds are
shortened causing an omega shape
252
laryngomalacia presentation is with
harsh whistling sound; inspiratory stridor
253
management of laryngomalacia is through
child development (grow out of it)
254
whooping cough is caused by
bordetella pertussis
255
bordella pertussis is what type of bacteria?
gram negative
256
is there a vaccination for pertussis?
yes
257
presentation of pertussis ?
mild coryzal symptoms, low grade fever, severe coughing fits post 1 week (paroxysmal cough) with inspiratory whoop post fit.
258
complications of coughing fits from whooping cough?
pneumothorax, faint or vomit. long term risk of bronchiectasis
259
infants may present differently with pertussis with instead
apnoeas
260
diagnosis of whooping cough is with
nasopharyngeal swab, PCR or bacterial culture.
261
management of whooping cough requires
supportive care and notifying public health. macrolide antibiotics and prophylactic antibiotics for contacts.
262
chronic lung disease of prematurity is also known as
bronchopulmonary dysplasia
263
chronic lung disease of prematurity occurs usually with those born before
28 weeks gestation
264
those born with chronic lung disease of prematurity usually require
oxygen therapy or intubation and ventilation at birth
265
Dx of chronic lung disease of prematurity
CXR
266
chronic lung disease of prematurity features
```
Low oxygen saturations
Increased work of breathing
Poor feeding and weight gain
Crackles and wheezes on chest auscultation
Increased susceptibility to infection
```
267
chronic lung disease of prematurity prevention is through
corticosteroids prior to birth, post caffeine, not over oxygenating and CPAP
268
CF is what sort of genetic condition?
autosomal recessive.
269
CF is a mutation of the
cystic fibrosis transmembrane conductance regulatory gene on chromosome 7
270
CF common mutation is the
delta-F508
271
CFTR gene in Cf codes for
cellular channels, particularly a type of chloride channel
272
fraction for carriers are
1/25
273
fraction of kids born with CF is
1/2500
274
consequences of CF includes
Thick pancreatic and biliary secretions, Low volume thick airway secretions, Congenital bilateral absence of the vas deferens
275
what pancreatic enzyme is missing in CF
pancreatic lipase
276
both parents are healthy, one sibling has cystic fibrosis and a second child does not have the disease, what is the likelihood of the second child being a carrier?
two in three.
277
CF is screened for at birth using
newborn bloodspot test.
278
first sign of CF is
Meconium ileus (thick and sticky bowel obstruction)
279
symptoms of CF are
```
Chronic cough
Thick sputum production
Recurrent respiratory tract infections
(steatorrhoea)
Abdominal pain and bloating
child tastes particularly salty sweat
(failure to thrive)
```
280
signs of CF
```
Low weight or height on growth charts
Nasal polyps
Finger clubbing
Crackles and wheezes on auscultation
Abdominal distention
```
281
during pregnancy CF may be tested for through
amniocentesis or chorionic villous sampling
282
sweat test requires
pilocarpine application and electrodes, then measure chloride concentration.
283
sweat test diagnostic point
>60 mmol/l
284
key colonisers in CF is
staph aureus and pseudomonas
285
Staph aureus in CF is prevented with
prophylactic flucloxacillin
286
Pseudomonas in Cf can be attempted to treat with
long term nebulised antibiotics such as tobramycin or Oral ciprofloxacin
287
management of Cf includes
Chest PT, exercise, high calorie diet, CREON tablets (enzyme replacement), flucloxacillin, bronchodilators, nebulised DNase and hypertonic saline and vaccinations
288
other treatments for Cf include
organ transplant, fertility treatment and genetic counselling.
289
patients with CF may require monitoring for
diabetes, osteoporosis, vitamin D deficiency and liver failure.
290
life expectancy median in Cf is
47 years
291
kartagner's syndrome refers too
primary ciliary dyskinesia
292
primary ciliary dyskinesia genetic spread is
autosomal recessive
293
key risk factor for primary ciliary dyskinesia is
consanguinity
294
kartagner's triad is
Paranasal sinusitis
Bronchiectasis
Situs Inversus
295
situs inversus refers too
internal (visceral) organs are mirrored inside the body
296
when only the heart is reversed it is called
dextrocardia
297
Dx of primary ciliary dyskinesia is
sample of the ciliated epithelium via nasal brushing or bronchoscopy.
298
Mx of primary ciliary dyskinesia is through
daily physiotherapy, a high calorie diet and antibiotics.
299
medical causes of abdominal pain
```
Constipation is also very common
Urinary tract infection
Coeliac disease
Inflammatory bowel disease
Irritable bowel syndrome
Mesenteric adenitis
Abdominal migraine
Pyelonephritis
Henoch-Schonlein purpura
Tonsilitis
Diabetic ketoacidosis
Infantile colic
```
300
common causes of abdominal pain in girls
```
Dysmenorrhea (period pain)
Mittelschmerz (ovulation pain)
Ectopic pregnancy
Pelvic inflammatory disease
Ovarian torsion
Pregnancy
```
301
surgical causes of pain in kids
appendicitis
intussuception
bowel obstruction
testicular torsion
302
red flags of abdominal pain
```
Persistent or bilious vomiting
Severe chronic diarrhoea
Fever
Rectal bleeding
Weight loss or faltering growth
Dysphagia (difficulty swallowing)
Nighttime pain
Abdominal tenderness
```
303
abdominal migraine refers too
episodes of central abdominal pain lasting more than 1 hour but with normal examination
304
abdominal migraine presentation
```
Nausea and vomiting
Anorexia
Pallor
Headache
Photophobia
Aura
```
305
treatment of acute abdominal migraine
Low stimulus environment (quiet, dark room)
Paracetamol
Ibuprofen
Sumatriptan
306
main preventative drug for abdominal migraine is
Pizotifen
307
Pizotifen is
serotonin agonist
308
secondary causes of constipation include
Hirschsprung’s disease, cystic fibrosis or hypothyroidism.
309
encopresis refers too
faecal incontinence
310
signs of constipation
abdominal pain, overflow soiling, straining painful stool and retentive posturing.
311
faecal incontinence is no pathological until over the age of
4 years
312
faecal impaction (rectally retained faeces) leads too
desensitisation of the rectum.
313
red flags for constipation
not passing meconium, neuro signs, vomiting, ribbon stools, abnormal anus or buttocks, failure to thrive or pain and bloating.
314
nice recommends for constipation
laxatives, high fibre diet, hydration, disimpaction regime and bowel diary.
315
first line laxative in constipation is
movicol
316
signs of a problematic reflux include
```
Chronic cough
Hoarse cry
Distress, crying or unsettled after feeding
Reluctance to feed
Pneumonia
Poor weight gain
```
317
red flags for vomiting include
not keeping down any feed, projectile vomiting, bile stained, haematemesis, abdominal distension, reduced consciousness, respiratory symptoms, blood in stools, rash, angiodema, signs of allergy, apnoeas and infection.
318
practical advice for reflux is
Small, frequent meals
Burping regularly to help milk settle
Not over-feeding
Keep the baby upright after feeding
319
problematic cases of reflux can be treated with
Gaviscon mixed with feeds
Thickened milk or formula (specific anti-reflux formulas are available)
Ranitidine
Omeprazole
320
severe causes of reflux may require
barium meal and endoscopy and rarely Surgical fundoplication
321
Sandifer’s Syndrome refers too
brief episodes of abnormal movements associated with gastro-oesophageal reflux
322
key features of sandifer's syndrome is
torticollis and dystonia
323
torticollis refers too
forceful contraction of the neck muscles causing twisting of the neck
324
dystonia refers too
abnormal muscle contractions causing twisting movements, arching of the back or unusual postures
325
West syndrome refers too
infantile spasms
326
features of pyloric stenosis are
failure to thrive, projectile vomiting, firm mass "large olive", visible peristalsis
327
blood gas analysis of pyloric stenosis will reveal
hypochloric (low chloride) metabolic alkalosis
328
Dx of pyloric stenosis is through
abdominal US
329
treatment of pyloric stenosis is
laparoscopic pyloromyotomy (known as “Ramstedt’s operation“)
330
viral causes of diarrhoea include
rotavirus and norovirus
331
what virus causes subacute diarrhoea?
adenovirus
332
what bacteria produces shiga toxin
E. coli 0157 and shigella
333
shiga toxin from E. coli 0157 may cause
haemolytic uraemic syndrome
334
commonest cause of traveller's diarhroea is
campylobacter jejuni
335
campylobacter is what sort of bacteria?
curved or spiral gram negative
336
spread of campylobacter is through
Raw or improperly cooked poultry
Untreated water
Unpasteurised milk
337
symptoms of campylobacter is
Abdominal cramps
Diarrhoea often with blood
Vomiting
Fever
338
incubation of campylobacter is
2-5 days
339
antibiotic choices for campylobacter are
azithromycin or ciprofloxacin.
340
antibiotic treatment for shigella is
azithromycin or ciprofloxacin.
341
shigella causes
bloody diarrhoea, abdominal cramps and fever for usually 1 week
342
salmonella incubation is for
12 hours to 3 days
343
symptoms of salmonella is
diarrhoea that can be associated with mucus or blood, abdominal pain and vomiting.
344
severe cases of salmonella treatment should be guided by
stool culture
345
patient develops symptoms of gastroenteritis soon after eating leftover fried rice that has been left at room temperature. It has a short incubation period after eating the rice before symptoms occur, and they recover within 24 hours. what was the causitive bacteria?
bacillus cereus
346
bacillus cereus type of bacteria?
gram positive rod
347
bacillus cereus toxin is called
cereulide
348
symptoms of bacillus cereus are
abdominal cramping and vomiting within 5 hours of ingestion and watery diarrhoea
349
Yersinia is an example of what sort of bacteria?
gram negative bacillus
350
what animal is a key carrier of yersinia
pigs
351
yersinia symptoms in children are
watery or bloody diarrhoea, abdominal pain, fever and lymphadenopathy.
352
older children with yersinia entercolitica may present with
ight sided abdominal pain due mesenteric lymphadenitis (inflammation in the intestinal lymph nodes) and fever.
353
Staph aureus can produce what type of toxin?
enterotoxin.
354
giardia lamblia is a
microscopic parasite
355
giardia lamblia lives in the
small intestine of mammals
356
giardia lamblia spreads via the
faecal oral transmission
357
diagnosis and treatment of giardia lamblia is via
stool microscopy and metronidazole.
358
principles of gastroenteritis management
barrier nursing, infection control, fluid challenge or rehydration solutions
359
post gastroenteritis complications include
Lactose intolerance
Irritable bowel syndrome
Reactive arthritis
Guillain–Barré syndrome
360
coeliac disease refers to
an autoimmune condition triggered by exposure to gluten
361
coeliac auto antibodies target
epithelial cells of the intestine
362
key coeliac antibodies to remember are
anti-tissue transglutaminase (anti-TTG) and anti-endomysial (anti-EMA).
363
coeliac disease particularly causes atrophy and inflammation of the
intestinal villi of the jejunum
364
symptoms of coeliac disease may include
```
Failure to thrive in young children
Diarrhoea
Fatigue
Weight loss
Mouth ulcers
Anaemia secondary to iron, B12 or folate deficiency
Dermatitis herpetiformis
```
365
genetic association with coeliac disease is
HLA-DQ2 gene
366
what type of antibody is anti-TTG and anti-EMA
IgA
367
diagnostic gold standard of coeliac disease is through
biopsy of the jejenum revealing crypt hypertrophy and villous atrophy
368
features of Crohn's disease
N – No blood or mucus (these are less common in Crohns.)
E – Entire GI tract
S – “Skip lesions” on endoscopy
T – Terminal ileum most affected and Transmural (full thickness) inflammation
S – Smoking is a risk factor (don’t set the nest on fire)
369
features of ulcerative colitis
```
C – Continuous inflammation
L – Limited to colon and rectum
O – Only superficial mucosa affected
S – Smoking is protective
E – Excrete blood and mucus
U – Use aminosalicylates
P – Primary sclerosing cholangitis
```
370
presentation of IBD is
perfuse diarrhoea, abdominal pain, bleeding, weight loss or anaemia. They may be systemically unwell during flares, with fevers, malaise and dehydration.
371
extra intestinal manifestations of IBD include
```
Finger clubbing
Erythema nodosum
Pyoderma gangrenosum
Episcleritis and iritis
Inflammatory arthritis
Primary sclerosing cholangitis (ulcerative colitis)
```
372
testing for IBD includes
faecal calprotectin, endoscopy, CT, MRI, biopsy (gold standard) and CRP with FBC, U+E, TSH and LFT
373
first line for inducing remission for crohn's is through
steroids
374
Crohn's specialist treatment for inducing remission is through
```
Azathioprine
Mercaptopurine
Methotrexate
Infliximab
Adalimumab
```
375
maintaining remission for crohn's disease is through first line
Azathioprine
| Mercaptopurine
376
surgical options for crohn's disease
resect if limited to distal ileum, or treat strictures and fistulae's.
377
inducing remission for ulcerative colitis is through first line
aminosalicylate (e.g. mesalazine oral or rectal)
378
second line for mild UC remission treatment
corticosteroids (e.g. prednisolone)
379
maintaining remission in UC is through
Aminosalicylate (e.g. mesalazine oral or rectal)
Azathioprine
Mercaptopurine
380
surgical options for UC include
panproctocolectomy with permanent ileostomy or ileo-anal anastomosis (J-pouch)
381
biliary atresia refers too
narrow or absent bile duct
382
biliary atresia results in
cholestasis
383
biliary atresia prevents the excretion of
conjugated bilirubin.
384
biliary atresia presents with
significant jaundice due to high conjugated bilirubin levels
385
suspect biliary atresia in children with
babies with a persistent jaundice, lasting more than 14 days in term babies and 21 days in premature babies
386
initial investigation for biliary atresia is with
is conjugated and unconjugated bilirubin.
387
majority of cases with neonatal jaundice are due to
benign breast milk jaundice
388
management of biliary atresia is through
“Kasai portoenterostomy
389
Kasai portoenterostomy refers too
attaching a section of the small intestine to the opening of the liver, where the bile duct normally attaches
390
long term biliary atresia commonly requires
full liver transplant
391
presentation of intestinal obstruction
Persistent vomiting. This may be bilious, containing bright green bile.
Abdominal pain and distention
Failure to pass stools or wind
Abnormal bowel sounds. These can be high pitched and “tinkling” early in the obstruction and absent later.
392
diagnosis of intestinal obstruction
AXR
393
abdominal AXR of obstruction reveals
dilated loops of bowel, collapsed loops of bowel distal to the obstruction and absence of air in the rectum.
394
management of intestinal obstruction requires
nil by mouth, nasogastric tube to drain the stomach and IV fluids.
395
congenital condition with the absence of bowel and rectal nerves refer too
Hirschsprung’s disease
396
myenteric plexus is also known as
Auerbach’s plexus
397
myenteric plexus is responsible for
peristalsis
398
the key pathophysiology of hirschsprung's disease is
absence of parasympathetic ganglion cells from the myenteric plexus due to failure of migration from proximal to distal gut
399
what occurs to the aganglionic bowel in hirschsprung's disease
constriction.
400
syndromes related to hirschsprung's disease
Downs syndrome
Neurofibromatosis
Waardenburg syndrome
Multiple endocrine neoplasia type II
401
Waardenburg syndrome refers too
(a genetic condition causing pale blue eyes, hearing loss and patches of white skin and hair)
402
presentation of hirschprung's disease is
```
Delay in passing meconium (more than 24 hours)
Chronic constipation since birth
Abdominal pain and distention
Vomiting
Poor weight gain and failure to thrive
```
403
hat life threatening complication is there of hirschsprung's disease?
Hirschsprung-Associated Enterocolitis which can lead too toxic megacolon and perforation of the bowel.
404
Hirschsprung-Associated Enterocolitis presents
within 2-4 weeks of birth with distension and diarrhoea with blood and septic signs
405
treatment of Hirschsprung-Associated Enterocolitis is
antibiotics, fluid resuscitation and decompression
406
diagnosis of Hirschsprung's disease is through
rectal biopsy
407
definitive treatment of Hirschsprung's is
surgical removal of aganglionic section of bowel.
408
intussusception commonly occurs between what ages
It typically occurs in infants 6 months to 2 years and is more common in boys.
409
associated conditions with intussusception
```
Concurrent viral illness
Henoch-Schonlein purpura
Cystic fibrosis
Intestinal polyps
Meckel diverticulum
```
410
presentation of intussusception
```
Severe, colicky abdominal pain
Pale, lethargic and unwell child
“Redcurrant jelly stool”
Right upper quadrant mass on palpation. This is described as “sausage-shaped”
Vomiting
Intestinal obstruction
```
411
diagnosis of intussusception is through
US or contrast enema
412
treatment of intussusception is with
surgical reduction, or therapeutic enema
413
peak incidence of appendicitis is between ages of
10 to 20 years old.
414
key presentation of appendicitis is
his typically starts as central abdominal pain, that moves down to the right iliac fossa (RIF)
415
McBurney's point refers too
localised area one third the distance from the anterior superior iliac spine (ASIS) to the umbilicus.
416
appendicitis Rovsing's sign is
palpation of the left iliac fossa causes pain in the RIF)
417
Rebound tenderness and percussion tenderness suggest
peritonitis, caused by a ruptured appendix.
418
other symptoms of appendicitis include
Loss of appetite (anorexia)
| Nausea and vomiting and guarding.
419
diagnosis of appendicitis is through
inflammatory markers, CT, US then a diagnostic laparoscopy.
420
differentials of appendicitis
ovarian cysts, meckel's diverticulum, mesenteric adenitis, appendix mass
421
type 1 diabetes may be caused by what viruses?
Coxsackie B virus and enterovirus
422
ideal blood glucose is between?
between 4.4 and 6.1 mmol/l.
423
Baby UTI presentation?
```
Fever
Lethargy
Irritability
Vomiting
Poor feeding
Urinary frequency
```
424
signs and symptoms of UTI in children?
```
Fever
Abdominal pain, particularly suprapubic pain
Vomiting
Dysuria (painful urination)
Urinary frequency
Incontinence
```
425
acute pyelonephritis presentation is through
A temperature greater than 38°C
| Loin pain or tenderness
426
Ix of UTI is through
clean catch urine with evidence of leukocytes and nitrites and a Midstream urine sample with culture and sensitivity testing.
427
children under 3 months with a fever should receive
IV antibiotics (e.g. ceftriaxone) and have a full septic screen, including blood cultures, bloods and lactate. A lumbar puncture should also be considered.
428
typical antibiotics for a UTI are
Trimethoprim
Nitrofurantoin
Cefalexin
Amoxicillin
429
all children under 6 months with a UTI should also receive a
US
430
all children with recurrent UTI's should receive a US within
6 weeks
431
damage from recurrent UTI's should receive what post 4-6 months to asses the damage?
DMSA scan
432
DMSA scan requires
gamma camera to assess how well the material is taken up by the kidneys. to indicate scarring
433
what condition predisposes to upper UTI's
Vesico-ureteric reflux
434
Vesico-ureteric reflux is diagnosed through
micturating cystourethrogram (MCUG).
435
micturating cystourethrogram (MCUG). should be used to investigate recurrent UTI's in those under the age of
<6 months or if FH present of VUR
436
micturating cystourethrogram (MCUG). involves
It involves catheterising the child, injecting contrast into the bladder and taking a series of xray films
437
vulvovaginitis presents as
```
Soreness
Itching
Erythema around the labia
Vaginal discharge
Dysuria (burning or stinging on urination)
Constipation
```
438
urine dipstick of a vulvovaginitis will show
leukocytes but no nitrates
439
nephrotic syndrome occurs when
the basement membrane in the glomerulus becomes highly permeable to protein,
440
nephrotic syndrome is common between the ages of
of 2 and 5 years.
441
the classic triad of nephrotic syndrome is
Low serum albumin
High urine protein content (>3+ protein on urine dipstick)
Oedema
442
other features that appear alongside nephrotic syndrome
Deranged lipid profile, with high levels of cholesterol, triglycerides and low density lipoproteins
High blood pressure
Hyper-coagulability
443
most common cause of nephrotic syndrome in children under the age of ten is
minimal change disease
444
secondary causes of nephrotic syndrome secondary to intrinsic kidney disease includes
Focal segmental glomerulosclerosis
| Membranoproliferative glomerulonephritis
445
systemic illnesses that may also cause nephrotic syndrome include
Henoch schonlein purpura (HSP)
Diabetes
Infection
446
renal biopsy and microscopy of minimal change disease will show
usually no abnormality
447
urinalysis of minimal change disease will usually show
small molecular weight proteins and hyaline casts.
448
management of minimal change disease is with
corticosteroids
449
2 – 5 year old child with oedema, proteinuria and low albumin, what is the underlying cause?
nephrotic syndrome.
450
nephrotic syndrome treatment is with
High dose steroids (i.e. prednisolone)
Low salt diet
Diuretics may be used to treat oedema
Albumin infusions may be required in severe hypoalbuminaemia
Antibiotic prophylaxis may be given in severe cases
451
alternative treatment for steroid resistant nephrotic syndrome is
ACE inhibitors and immunosuppressants
452
nephritis causes
Reduction in kidney function
Haematuria: invisible or visible amounts of blood in the urine
Proteinuria: although less than in nephrotic syndrome
453
commonest causes of nephritis is
post-streptococcal glomerulonephritis and IgA nephropathy (Berger’s disease).
454
Post-Streptococcal Glomerulonephritis occurs
1 – 3 weeks after a β-haemolytic streptococcus infection, such as tonsillitis caused by Streptococcus pyogenes
455
pathophysiology of post streptococcal glomerulonephritis
Immune complexes made up of streptococcal antigens, antibodies and complement proteins get stuck in the glomeruli of the kidney and cause inflammation. This inflammation leads to an acute deterioration in renal function, causing an acute kidney injury.
456
Ix of Post-Streptococcal Glomerulonephritis is with
, positive throat swab results and anti-streptolysin antibody titres found on a blood test.
457
majority of patients with post streptococcal glomerulonephritis will recover but some will require
They may need treatment with antihypertensive medications and diuretics if they develop complications such as hypertension and oedema.
458
IgA Nephropathy is related too
Henoch-Schonlein Purpura, which is an IgA vasculitis
459
IgA nephropathy pathology
IgA deposits in the nephrons of the kidney causes inflammation
460
renal biopsy of IgA nephropathy will show
“IgA deposits and glomerular mesangial proliferation”.
461
IgA nephropathy usually presents in
teenagers or young adults.
462
Tx of IgA nephropathy is with
supportive treatment of the renal failure and immunosuppressant medications such as steroids and cyclophosphamide
463
Haemolytic Uraemic Syndrome is triad of
Haemolytic anaemia, AKI and thrombocytopenia
464
management of HUS requires
Urgent referral to the paediatric renal unit for renal dialysis if required
Antihypertensives if required
Careful maintenance of fluid balance
Blood transfusions if required
465
enuresis refers too as
involuntary urination
466
most children develop control of daytime urination by
2 years
467
most children develop control of night time urination by
3-4 years
468
Primary nocturnal enuresis refers too
the child has never managed to be consistently dry at night.
469
causes of primary nocturnal enuresis
overactive bladder, fluid intake, failure to wake, psychological distress or chronic conditions
470
Secondary Nocturnal Enuresis refers too
wetting the bed when they have previously been dry for at least 6 months
471
causes of secondary nocturnal enuresis include
```
Urinary tract infection
Constipation
Type 1 diabetes
New psychosocial problems (e.g. stress in family or school life)
Maltreatment
```
472
nocturnal enuresis may be treated with
Desmopressin is an analogue of vasopressin (also known as anti-diuretic hormone).
473
over active bladder may be treated with
Oxybutinin
474
what form of polycystic kidney disease presents earlier in life
autosomal recessive polycystic kidney disease
475
Autosomal recessive polycystic kidney disease (ARPKD) is caused by a mutation of
polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6.
476
polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6 codes for
fibrocystin/polyductin protein complex (FPC)
477
fibrocystin/polyductin protein complex (FPC) is responsible for
the creation of tubules and the maintenance of healthy epithelial tissue in the kidneys, liver and pancreas.
478
underlying pathology of Autosomal recessive polycystic kidney disease (ARPKD) is
Cystic enlargement of the renal collecting ducts
Oligohydramnios, pulmonary hypoplasia and Potter syndrome
Congenital liver fibrosis
479
ARPKD usually presents in the antenatal period with
oligohydramnios and polycystic kidneys seen on antenatal scans
480
lack of amniotic fluid causes
Potter syndrome and pulmonary hypoplasia
481
Potter syndrome refers too
underdeveloped ear cartilage, low set ears, a flat nasal bridge and abnormalities of the skeleton
482
outcomes of polycystic kidney disease are
```
Liver failure due to liver fibrosis
Portal hypertension leading to oesophageal varices
Progressive renal failure
Hypertension due to renal failure
Chronic lung disease
```
483
survival rate to adulthood for polycystic kidney disease
1/3rd
484
Multicystic dysplastic kidney (MCDK) is diagnosed
on antenatal US
485
a child under the age of 5 years presenting with a mass in the abdomen. consider
Wilm's tumour
486
Wilm's tumour presentation
```
Abdominal pain
Haematuria
Lethargy
Fever
Hypertension
Weight loss
```
487
diagnosis of wilm's tumour is through
US then CT or MRI to stage and definitively biopsy with histology
488
management of wilm's tumour is with
nephrectomy with adjuvant chemo or RT
489
posterior urethral valve is a cause of
hydronephrosis
490
posterior urethral valve presents with
```
Difficulty urinating
Weak urinary stream
Chronic urinary retention
Palpable bladder
Recurrent urinary tract infections
Impaired kidney function
```
491
posterior urethral valve ix
antenatal scans or abdo US, MCUG, cystoscopy which can also ablate the tissue.
492
Undescended testes increase risk of
testicular torsion, infertility and testicular cancer
493
undescended testes post 6 months will require
Orchidopexy
494
Hypospadias refers too
posterior urethral meatus
495
hydrocele is a collection of fluid within the
tunica vaginalis
496
tunica vaginalis can communicate with the peritoneal cavity via
the processus vaginalis
497
hydrocele presentation is
soft, smooth, non-tender swelling around one of the testes.
498
key feature of a hydrocele is that it
transilluminates with light
499
differentials for a hydrocele include
```
Hydrocele
Partially descended testes
Inguinal hernia
Testicular torsion
Haematoma
Tumours (rare)
```
500
surfactant is produced by
type 2 alveolar cells
501
type 2 alveolar cells mature around what weeks of gestation?
24 and 34 weeks
502
extended hypoxia during child birth may lead too
hypoxic-ischaemic encephalopathy (HIE),
503
hypoxic-ischaemic encephalopathy (HIE), may lead too
cerebral palsy
504
issues with neonatal resus
babies have a large surface area, they loos heat rapidly and risk of meconium inhalation.
505
principles of neonatal resus
```
warm the baby
calculate APGAR score
stimulate breathing
inflation breaths
and chest compressions
```
506
how best to stimulate breathing in a neonate in resus
neutral position to keep airway open with a towel under shoulders
507
cycle of breaths in neonate resus
Two cycles of five inflation breaths. No response the 30 seconds of ventilation breaths.
508
chest compression to breath ratio
3:1
509
APGAR score consists of
appearance, pulse, grimmace, activity, and respiration
510
score 0 on APGAR would be
blue baby, absent pulse, no response to stimulation, floppy and absent respirations.
511
APGAR score 2 for pulse would be
>100
512
max APGAR score is
10
513
delayed cord clamping is beneficial for
improved haemoglobin, iron stores and blood pressure and a reduction in intraventricular haemorrhage and necrotising enterocolitis
514
immediately afterbirth you should seek to
```
Skin to skin
Clamp the umbilical cord
Dry the baby
Keep the baby warm with a hat and blankets
Vitamin K
Label the baby
Measure the weight and length
```
515
Vit K to the baby is via a
IM injection
516
on day 5 what neonate screening test is under taken?
day 5
517
blood spot screening test screens for
```
Sickle cell disease
Cystic fibrosis
Congenital hypothyroidism
Phenylketonuria
Medium-chain acyl-CoA dehydrogenase deficiency (MCADD)
Maple syrup urine disease (MSUD)
Isovaleric acidaemia (IVA)
Glutaric aciduria type 1 (GA1)
Homocystin
```
518
results for a blood spot test take how many weeks to come back?
6-8 weeks
519
newborn examination is undertaken how many hours after birth?
72 hours
520
neonate sats should be measured as
pre ductal and post ductal
521
what would be considered pathological difference in ductal sats
>2%
522
pre ductal sats are measured in the
right hand
523
the right hand receives blood from the
right subclavian artery, a branch of the brachiocephalic artery, which branches from the aorta before the ductus arteriosus.
524
post ductal sats are measured from the
feet
525
red reflex is absent in
congenital cataracts and retinoblastoma.
526
single palmar crease is associated with
down's syndrome
527
moro reflex is the
when rapidly tipped backwards the arms and legs will extend
528
rooting reflex is the
tickling the cheek will cause them to turn towards the stimulus
529
stepping reflex is the
when held upright and the feet touch a surface they will make a stepping motion
530
talipes refer too
the ankles are in a supinated position, rolled inwards
531
Port wine stains refer too
pink patches of skin, often on the face, caused by abnormalities affecting the capillaries.
532
sturge weber syndrome refers too
visual impairment,port wine stains and learning difficulties, headaches, epilepsy and glaucoma.
533
examples of birth injures?
Caput Succedaneum, Cephalohaematoma, Facial Paralysis, Erbs Palsy and Fractured Clavicle
534
refers too
oedema collecting on the scalp outside the periosteum.
535
does caput succedeneum (oedema) cross the suture lines?
yes
536
the presence of a cephalohaematoma requires the monitoring for
anaemia, jaundice and resolution
537
Erb's palsy is damage to the
C5/C6 nerves in the brachial plexus during birth.
538
Erb's palsy presentation
Internally rotated shoulder
Extended elbow
Flexed wrist facing backwards (pronated)
Lack of movement in the affected arm
539
common organism for neonatal sepsis is
Group B streptococcus (GBS)
540
features of neonatal sepsis
```
Fever
Reduced tone and activity
Poor feeding
Respiratory distress or apnoea
Vomiting
Tachycardia or bradycardia
Hypoxia
Jaundice within 24 hours
Seizures
Hypoglycaemia
```
541
red flags for neonatal sepsis
Confirmed or suspected sepsis in the mother
Signs of shock
Seizures
Term baby needing mechanical ventilation
Respiratory distress starting more than 4 hours after birth
Presumed sepsis in another baby in a multiple pregnancy
542
for one risk factor of neonatal sepsis you should
monitor the observations and clinical condition for at least 12 hours
543
for two risk factors of neonatal sepsis you should
start antibiotics
544
first line antibiotics for neonatal sepsis are
benzylpenicillin and gentamycin
545
causes of Hypoxic-Ischaemic Encephalopathy
Maternal shock
Intrapartum haemorrhage
Prolapsed cord, causing compression of the cord during birth
Nuchal cord, where the cord is wrapped around the neck of the baby
546
Hypoxic-Ischaemic Encephalopathy grading is called
sarnat staging
547
Hypoxic-Ischaemic Encephalopathy mild features
Poor feeding, generally irritability and hyper-alert
Resolves within 24 hours
Normal prognosis
548
Hypoxic-Ischaemic Encephalopathy moderate features
Poor feeding, lethargic, hypotonic and seizures
Can take weeks to resolve
Up to 40% develop cerebral palsy
549
Hypoxic-Ischaemic Encephalopathy severe features
Reduced consciousness, apnoeas, flaccid and reduced or absent reflexes
Up to 50% mortality
Up to 90% develop cerebral palsy
550
managements of Hypoxic-Ischaemic Encephalopathy
MDT, supportive and therapeutic hypothermia
551
intention of therapeutic hypothermia is to
reduce the inflammation and neurone loss
552
physiological jaundice mechanism
fragile RBC's, less developed liver function
553
in preamture neonates the immature liver puts the neonates at risk of
kernicterus.
554
kernicterus is
brain damage due to high bilirubin levels
555
prolonged jaundice is classified as how long with full term babies?
14 days
556
prolonged jaundice is classified as how long in premature babies?
21 days
557
causes of prolonged jaundice in the neonate
biliary atresia, hypothyroidism and G6PD deficiency.
558
direct coombs test is for
haemolysis
559
neonatal jaundice may be corrected through
phototherapy, or sometimes exchange transfusion.
560
phototherapy mechanism
converts unconjugated bilirubin into isomers for urine and bile excretion without conjugation.
561
phototherapy rebound bilirubin should be measured
12-18 hours after stopping
562
kernicterus presents with
less responsive, floppy, drowsy baby with poor feeding.
563
apnoeas are defined as periods of
breathing stops spontaneously for more than 20 seconds, or shorter periods with oxygen desaturation or bradycardia.
564
apnoeas occurs due to immaturity of the
autonomic nervous system
565
apnoeas may be a sign of
```
Infection
Anaemia
Airway obstruction (may be positional)
CNS pathology, such as seizures or haemorrhage
Gastro-oesophageal reflux
Neonatal abstinence syndrome
```
566
apnoeas may be managed through
tactile stimulation, I.V caffeine and apnoea monitors
567
retinal blood vessel development starts around what weeks and is completed by
16 weeks and completed by 37 - 40 weeks
568
retinal blood vessel development is stimulated by
hypoxia
569
post delivery for a premature neonate after support and hypoxia returns what occurs with the retinal vessels?
excessive and scarred blood vessels that may regress or cause retinal detachment
570
zone 1 of the retina includes
optic nerve and the macula
571
zone 2 of the retina includes
ora serrata, the pigmented boarder between the retina and ciliary body
572
zone 3 of the retina includes
ora serrata
573
premature retinopathy plus disease refers too
tortuous vessels and hazy vitreous humour.
574
screening for premature retinopathy should occurs
for every baby <32W or <1.5kg and for every 2 weeks and can cease once the retinal vessels enter zone 3, usually at around 36 weeks gestation.
575
first line treatment for premature retinopathy
transpupillary laser photocoagulation
576
treatment for premature retinopathy alternatives
cryotherapy and injections of intravitreal VEGF inhibitors, Surgery
577
CXR or respiratory distress syndrome demonstrates
ground glass
578
presentation of Necrotising Enterocolitis
```
Intolerance to feeds
Vomiting, particularly with green bile
Generally unwell
Distended, tender abdomen
Absent bowel sounds
Blood in stools
```
579
Necrotising Enterocolitis Ix
FBC, CRP, capillary blood gas for metabolic acidosis, blood culture, AXR
580
AXR in necrotising enterocolitis demonstrates
dilated bowel, oedema, pneumatosis intestinalis (gas in bowel wall), pneumoperitoneum (free gas in peritoneal cavity), gas in portal veins.
581
necrotising enterocolitis Tx
nil by mouth with IV fluids, total parenteral nutrition (TPN) and antibiotics. surgical emergency.
582
necrotising enterocolitis carries what risk post op
Short bowel syndrome
583
CNS Signs of neonatal abstinence
```
Irritability
Increased tone
High pitched cry
Not settling
Tremors
Seizures
```
584
Vasomotor and respiratory Signs of neonatal abstinence:
Yawning
Sweating
Unstable temperature and pyrexia
Tachypnoea (fast breathing)
585
Metabolic and gastrointestinal: signs of neonatal abstinence
Poor feeding
Regurgitation or vomiting
Hypoglycaemia
Loose stools with a sore nappy area
586
management of neonatal abstinence syndrome
NAS chart for at least 3 days with urine samples
587
opiate withdrawal in neonates require
Oral morphine sulphate
588
non-opiate withdrawal in neonates require
Oral phenobarbitone
589
fetal alcohol syndrome presents as
```
Microcephaly (small head)
Thin upper lip
Smooth flat philtrum (the groove between the nose and upper lip)
Short palpebral fissure (short horizontal distance from one side of the eye and the other)
Learning disability
Behavioural difficulties
Hearing and vision problems
Cerebral palsy
```
590
congenital rubella syndrome presentation
Congenital cataracts
Congenital heart disease (PDA and pulmonary stenosis)
Learning disability
Hearing los
591
Congenital Toxoplasmosis presentation
Intracranial calcification
Hydrocephalus
Chorioretinitis
592
congenital cytomegalovirus presentation
```
Fetal growth restriction
Microcephaly
Hearing loss
Vision loss
Learning disability
Seizures
```
593
zika virus is spread by the
Aedes mosquitos
594
zika congenital syndrome
Microcephaly
Fetal growth restriction
Other intracranial abnormalities, such as ventriculomegaly and cerebellar atrophy
595
acceptable weight loss in babies are
breast fed babies to loose up to 10% and formula fed babies to loose up to 5% of their body weight by day 5
596
if a child is on the 91st centile for height this means?
she is taller than 91% of children her age.
597
three phases of growth are
First 2 years: rapid growth driven by nutritional factors
From 2 years to puberty: steady slow growth
During puberty: rapid growth spurt driven by sex hormones
598
overweight is defined as a BMI above what centile?
85th percentile
599
obese is defined as being above what centile?
above the 95th percentile
600
mid parental height =
(height of mum + height of dad) / 2.
601
initial investigations for faltering growth
```
Urine dipstick, for urinary tract infection
Coeliac screen (anti-TTG or anti-EMA antibodies)
```
602
boys predicated height formula
(mother height + fathers height + 14cm) / 2
603
girls predicated height formula
(mothers height + father height – 14cm) /
604
Constitutional Delay In Growth and Puberty refers too
short stature in childhood when compared with peers but normal height in adulthood
605
Constitutional Delay In Growth and Puberty key feature
delayed bone age
606
4 domains of child development
Gross motor
Fine motor
Language
Personal and social
607
gross motor 4 months
This starts with being able to support their head and keep it in line with the body
608
gross motor 6 months
They can keep their trunk supported on their pelvis (i.e. maintain a sitting position) by 6 months, however they often don’t have the balance to sit unsupported at this stage.
609
gross motor 9 months
They should sit unsupported by 9 months. They can start crawling at this stage. They can also keep their trunk and pelvis supported on their legs (i.e. maintain a standing position) and bounce on their legs when supported.
610
gross motor 12 months
They should stand and begin cruising (walking whilst holding onto furniture).
611
gross motor 15 months
Walk unaided.
612
gross motor 18 months
Squat and pick things up from the floor.
613
gross motor 2 years
Run. Kick a ball.
614
fine motor early milestones 6 months:
Palmar grasp of objects (wraps thumb and fingers around the object).
615
fine motor early milestones 9 months:
Scissor grasp of objects (squashes it between thumb and forefinger).
616
fine motor early milestones
Pincer grasp (with the tip of the thumb and forefinger).
617
fine motor early milestones 14-18 months
They can clumsily use a spoon to bring food from a bowl to their mouth
618
fine motor drawing skills 12 months
Holds crayon and scribbles randomly
619
fine motor skills tower of bricks 14 months
Tower of 2 bricks
620
fine motor skills tower of bricks 18 months
Tower of 4 bricks
621
fine motor skills pencil grasp under 2 years
Palmar supinate grasp (fist grip)
622
expressive language milestones month 3
Cooing noises
623
expressive language milestones month 9
Babbles, sounding more like talking but not saying any recognisable words
624
expressive language milestones month 12
Says single words in context, e.g. “Dad-da” or “Hi”
625
expressive language milestones month 18
Has around 5 – 10 words
626
expressive language milestones 2 years
Combines 2 words. Around 50+ words total.
627
receptive language milestones month 3
Recognises parents and familiar voices and gets comfort from these
628
receptive language milestones month 5
Listens to speech
629
receptive language milestones month 12
Follows very simple instructions
630
receptive language milestones 2 years
Understands verbs, for example “show me what you eat with”
631
personal and social milestones week 6
smiles
632
personal and social milestones month 3
Communicates pleasure
633
personal and social milestones month 12
Engages with others by pointing and handing objects. Waves bye bye. Claps hands.
634
personal and social milestones month 18
Imitates activities such as using a phone
635
not sitting unsupported is a red flag at what month?
12
636
not holding an object is a red flag at what month?
5
637
not standing independently is a red flag at what month?
18
638
not walking independently at how many years is a red flag?
2 years
639
global developmental delay differentials
```
Down’s syndrome
Fragile X syndrome
Fetal alcohol syndrome
Rett syndrome
Metabolic disorders
```
640
gross motor delay differentials
```
Cerebral palsy
Ataxia
Myopathy
Spina bifida
Visual impairment
```
641
fine motor delays differentials
```
Dyspraxia
Cerebral palsy
Muscular dystrophy
Visual impairment
Congenital ataxia
```
642
language delays differentials
```
Hearing impairment
Learning disability
Neglect
Autism
Cerebral palsy
```
643
personal and social delay differentials
Parenting issues
| Autism
644
dysgraphia refers too
refers to a specific difficulty in writing.
645
dyspraxia refers too
developmental co-ordination disorder
646
capacity requires you to demonstrate
understanding, retaining info, weighing up options and communication
647
klinefleter's syndrome chromosomes
XXY
648
turner's syndrome chromosomes
XO
649
legal framework for children safeguarding is
Children Act 1989
650
both parents are carriers of an autosomal recessive condition, what is the chance of the children having the condition?
have a 1 in 4 (or 25%) of having the disease,
651
one parent has an autosomal recessive disease, and the other parent is a carrier. what is the chance of the children being inflicted?
Therefore the children of these parents have a 50% chance of having the disease and a 50% chance of being a carrier.
652
both parents are healthy, one sibling has a single gene disease (e.g. cystic fibrosis) disease, a second child does not have the disease, what is the likelihood of the second child being a carrier.
the risk of the second child being a carrier is 2 in 3. this is because we know that they do not have the phenotype of the disease.
653
example of deletion disorder?
cri du chat, which is caused by a missing portion of chromosome 5
654
example of duplication disorder
Charcot-Marie-Tooth, which can be caused by a duplication of the short arm of chromosome 17.
655
translocation disorder example
“Philadelphia chromosome” translocation in acute myeloid leukaemia, which is a reciprocal translocation between chromosome 9 and chromosome 22.
656
acrocentric chromosome examples
13, 14, 15, 21 and 22. They have one long arm and one short arm.
657
Robertsonian translocation refers too
acrocentric chromosome loosing its short arm
658
patau syndrome is a
trisomy 13
659
patau syndrome causes
ysmorphic features, structural abnormalities affecting almost all areas of their body and learning disability. They have characteristic “rocker bottom feet”,
660
edward syndrome is
trisomy 18
661
edward syndrome causes
resulting in dysmorphic features and learning disability. They also have “rocker bottom feet”.
662
in sex cells male mitochondria is located in the
the tail
663
mitochondrial DNA inheritance comes from the
mother
664
down syndrome features
```
Hypotonia (reduced muscle tone)
Brachycephaly (small head with a flat back)
Short neck
Short stature
Flattened face and nose
Prominent epicanthic folds
Upward sloping palpable fissures
Single palmar crease
```
665
complications for down syndrome
Learning disability
Recurrent otitis media
Deafness. Eustachian tube, Visual problems, Hypothyroidism, Cardiac defects, Atlantoaxial instability, Leukaemia, Dementia
666
combined test screening for down's biochemical results that indicate high risk
BHCG high, PAPPA low
667
klinefelter syndrome features
```
Taller height
Wider hips
Gynaecomastia
Weaker muscles
Small testicles
Reduced libido
Shyness
Infertility
Subtle learning difficulties
```
668
klinefelter syndrome treatment
Testosterone injections
Advanced IVF techniques
Breast reduction surgery +
MDT
669
features of Turner's syndrome
```
Short stature
Webbed neck
High arching palate
Downward sloping eyes with ptosis
Broad chest with widely spaced nipples
Cubitus valgus
Underdeveloped ovaries with reduced function
Late or incomplete puberty
Most women are infertile
```
670
cubitus valgus refers too
arm is extended downwards with the palms facing forward, the angle of the forearm at the elbow is exaggerated, angled away from the body.
671
management of turner's syndrome involves
GH therapy, oestrogen, progesterone and fertility treatment
672
noonan syndrome transmission
autosomal dominant
673
features of noonan syndrome
```
Short stature
Broad forehead
Downward sloping eyes with ptosis
Hypertelorism (wide space between the eyes)
Prominent nasolabial folds
Low set ears
Webbed neck
Widely spaced nipples
```
674
noonan syndrome associated conditions
Congenital heart disease, Cryptorchidism, Learning disability
Bleeding disorders
Lymphoedema, increased risk of leukaemia and neuroblastoma
675
marfan syndrome genetic transmission
autosomal dominant
676
pathology of marfan
affecting the gene responsible for creating fibrillin
677
features of marfan syndrome
```
Tall stature
Long neck
Long limbs
Long fingers (arachnodactyly)
High arch palate
Hypermobility
Pectus carinatum or pectus excavatum
Downward sloping palpable fissures
```
678
associated conditions with marfan
Lens dislocation in the eye
Joint dislocations and pain due to hypermobility
Scoliosis of the spine
Pneumothorax
Gastro-oesophageal reflux
Mitral valve prolapse (with regurgitation)
Aortic valve prolapse (with regurgitation)
Aortic aneurysms
679
meet someone with tall, has hypermobility or a murmur suggestive of mitral or aortic regurgitation what do you think of?
marfan syndrome
680
management of marfan's involves
surgical correction of aortic aneurysms, avoiding stimulants and intense exercise, betablockers and ARB's, PT, genetic counselling and yearly ECHO
681
features of fragile x syndrome
```
Intellectual disability
Long, narrow face
Large ears
Large testicles after puberty
Hypermobile joints (particularly in the hands)
Attention deficit hyperactivity disorder (ADHD)
Autism
Seizures
```
682
prada willi syndrome arises from
deletion mutation of the proximal arm of chromosome 15 from the father
683
features of prada willi syndrome
Constant insatiable hunger that leads to obesity
Poor muscle tone as an infant (hypotonia)
Mild-moderate learning disability
Hypogonadism
Fairer, soft skin that is prone to bruising
Mental health problems, particularly anxiety
Dysmorphic features
Narrow forehead
Almond shaped eyes
Strabismus
Thin upper lip
Downturned mouth
684
NICE indicates for prada willi syndrome
Growth hormone to imrpove muscle and body composition
685
angelman syndrome is caused by the loss of which gene
UBE3A gene
686
angelman syndrome is due to a deletion on which chromosome
chromosome 15
687
features of angelman syndrome
```
Delayed development and learning disability
Severe delay or absence of speech development
Coordination and balance problems (ataxia)
Fascination with water
Happy demeanour
Inappropriate laughter
Hand flapping
Abnormal sleep patterns
Epilepsy
Attention-deficit hyperactivity disorder
Dysmorphic features
Microcephaly
Fair skin, light hair and blue eyes
Wide mouth with widely spaced teeth
```
688
william syndrome is caused by deletion on what chromosome?
7
689
features of william syndrome ?
```
Broad forehead
Starburst eyes (a star-like pattern on the iris)
Flattened nasal bridge
Long philtrum
Wide mouth with widely spaced teeth
Small chin
Very sociable trusting personality
Mild learning disability
```
690
associated conditions with william syndrome
Supravalvular aortic stenosis (narrowing just above the aortic valve)
Attention-deficit hyperactivity disorder
Hypertension
Hypercalcaemia
691
features of syncope that differentiate it from a seizure
lightheaded, prolonged upright, sweating, blurring of vision, reduced tone, return to consciousness shortly after falling, no prolonged post-ictal period
692
generalised tonic clonic seizures associated with
tongue biting, incontinence, groaning and irregular breathing.
693
first line for generalised tonic-clonic seizure
sodium valproate
694
second line for generalised tonic clonic seizure
lamotrigine or carbamazepine
695
focal sezirues start in the
temporal lobes
696
focal seizures present with
Hallucinations
Memory flashbacks
Déjà vu
Doing strange things on autopilot
697
first line focal seizure treatment is
carbamazepine or lamotrigine
698
second line for focal seizures are
sodium valproate or levetiracetam
699
first line absence seizures treatment
sodium valproate or ethosuximide
700
atonic seizures a characterised by
They are characterised by brief lapses in muscle tone.
701
atonic seizure first line
sodium valproate
702
myoclonic seizure refers too
sudden brief muscle contractions, like a sudden “jump”.
703
myoclonic seizure first line is
sodium valproate
704
infantile spasms "west syndrome" prognosis
1/ die by 25, 1/3rd seizure free
705
infantile spasms treatment
Prednisolone
| Vigabatrin
706
EEG as an Ix for seizure occurs usually
after second presentation unless atypical
707
MRI for epilepsy should be considered when
The first seizure is in children under 2 years
Focal seizures
There is no response to first line anti-epileptic medications
708
additional investigations to consider for epilepsy
ECG, blood electrolytes, blood glucose, blood cultures, urine cultures and lumbar punctures
709
sodium valproate mechanism
by increasing the activity of GABA,
710
SE of sodium valproate
Teratogenic, so patients need careful advice about contraception
Liver damage and hepatitis
Hair loss
Tremor
711
SE of carbamazepine
Agranulocytosis
Aplastic anaemia
Induces the P450 system so there are many drug interactions
712
SE of lamotrigine
Stevens-Johnson syndrome or DRESS syndrome. These are life threatening skin rashes.
Leukopenia
713
status epilepticus is defined as
lasting more than 5 minutes or more than 3 seizures in one hour.
714
status epileptics management
Secure the airway
Give high-concentration oxygen
Assess cardiac and respiratory function
Check blood glucose levels
Gain intravenous access (insert a cannula)
IV lorazepam, repeated after 10 minutes if the seizure continues
715
Cyanotic breath holding spells occur
After letting out a long cry they stop breathing, become cyanotic and lose consciousness. Within a minute they regain consciousness and start breathing.
716
reflexic anoxic seizure is when
the child is startled. The vagus nerve sends strong signals to the heart that causes it to stop beating. The child will suddenly go pale, lose consciousness and may start to have some seizure-like muscle twitching. Within 30 seconds the heart restarts and the child becomes conscious again.
717
features of a migraine that are different to a tension type headache
Unilateral
More severe
Throbbing in nature
Take longer to resolve
718
migraines are associated with
Visual aura
Photophobia and phonophobia
Nausea and vomiting
Abdominal pain
719
managment of migraines in children require
```
Rest, fluids and low stimulus environment
Paracetamol
Ibuprofen
Sumatriptan
Antiemetics, such as domperidone
```
720
migraine prophylaxis includes
propranolol, pizotifen, topiramate
721
when an adult patient presents with a migraine ask about what?
recurrent central abdominal pain as a child. They may have a history of abdominal migraine that started before the headaches.
722
spastic hypertonia cerebral palsy refers too
(increased tone) and reduced function resulting from damage to upper motor neurones
723
dyskinetic cerebral palsy refers too
problems controlling muscle tone, with hypertonia and hypotonia, causing athetoid movements and oro-motor problems. This is the result of damage to the basal ganglia.
724
ataxic cerebral palsy refers too
problems with coordinated movement resulting from damage to the cerebellum
725
hemiplegic gait indicates
indicates an upper motor neurone lesion
726
ataxic gait indicates
cerebellar lesion
727
high stepping gait indicates
indicates foot drop or a lower motor neurone lesion
728
waddling gait indicates
indicates pelvic muscle weakness due to myopathy
729
upper motor neurone signs
muscle bulk preserved, hypertonia, slightly reduced poer and brisk reflexes
730
lower motor neurone signs
reduced muscle bulk, fasiculations, hypotonia, reduced power and reduced reflexes
731
people with cerebral palsy often have what signs
upper motor neurone signs
732
paediatrician my use what drugs for those with cerebral palsy
muscle relaxants, anti-epileptics and glycopyrronium bromide for excessive drooling
733
misalignment of the eyes is referred to as
strabismus
734
strabismus causes
double vision
735
concomitant squints are due to
differences in the control of the extra ocular muscles.
736
Amblyopia refers too
the affected eye becomes passive and has reduced function compared to the other dominant eye
737
Hirschberg’s test demonstrates
Deviation from the centre of a pen torch reflecting off the cornea will indicate a squint.
738
Tx for squints in kids are
occlusive patch or atropine drops in the good eye
739
CSF is created in each ventricle by
choroid plexuses
740
CSF is absorbed by what to get into the venous sytem
arachnoid granulations.
741
Arnold-Chiari malformation is where
cerebellum herniates downwards through the foramen magnum, blocking the outflow of CSF
742
signs of hydrocephalus in kids are
increasing occipito-frontal circumference, Bulging anterior fontanelle
Poor feeding and vomiting
Poor tone
Sleepiness
743
mainstay treatment for hydrocephalus is
Ventriculoperitoneal Shunt
744
craniosynostosis is the process of
the skull sutures close prematurely leading to increasing intracranial pressure
745
plagiocephaly means
flattening of one area of the baby’s head
746
brachycephaly refers too
refers to flattening at the back of the head
747
what should you look for and what should you exclude when managing Plagiocephaly
exclude craniosynotosis, and assess for congenital muscular torticollis
748
If there is a 5 year old boy presenting with vague symptoms of muscle weakness and the description is that you notice them using their hands on their legs to help them stand up. what is the condition and underlying mechanism?
Duchenne's muscular dystrophy and x linked recessive
749
Gower's sign is
To stand up, they get onto their hands and knees, then push their hips up and backwards like the “downward dog” yoga pose. They then shift their weight backwards and transfer their hands to their knees. Whilst keeping their legs mostly straight they walk their hands up their legs to get their upper body erect.
750
duchennes muscular dystrophy is caused by a defect in the what gene?
dystrophin
751
what has been used to slow progression of duchennes muscular dystrophy
oral steroids and creatine supplementals
752
features of myotonic dystrophy
Progressive muscle weakness
Prolonged muscle contractions
Cataracts
Cardiac arrhythmias
753
Emery-Dreifuss muscular dystrophy usually presents in childhood with
contractures, most commonly in the elbows and ankles.
754
classic initial symptoms of Facioscapulohumeral Muscular Dystrophy
A classic initial symptom is sleeping with their eyes slightly open and weakness in pursing their lips
