Paediatrics Flashcards

(913 cards)

1
Q

what are 3 characteristic signs of pneumonia on examination?

A

bronchial breath sounds
focal coarse crackles
dullness to percussion

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2
Q

what is the most common cause of pneumonia in children?

A

streptococcus pneumonia

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3
Q

what is the most common viral cause of pneumonia?

A

respiratory syncytial virus (RSV)

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4
Q

what is the typical x-ray finding in s. aureus pneumonia?

A

pneumatocoeles (round air filled cavities) and multilobe consolidation

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5
Q

what are 2 bacteria that are more likely to cause pneumonia in pre/un-vacinated children?

A

GBS
Haemophilus influenza

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6
Q

which bacterial pneumonia can cause extrapulmonary manifestations and what are they?

A

Mycoplasma pneumonia
Can cause erythema multiforme

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7
Q

what is the management of pneumonia?

A

1st - amoxicillin

+/- macrolide (erythro, clarithro, azithro) for atypicals or allergy

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8
Q

what is the name of the condition where people cannot convert IgM to IgG so cannot form long term immunity?

A

immunoglobulin class-switch recombination deficiency

VACCINES DONT WORK!

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9
Q

what age group is typically affected by croup?

A

6 months - 2years

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10
Q

what is the pathophysiology of croup?

A

upper resp tract Ix causing oedema of the larynx

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11
Q

what is the most common pathogenic cause of croup?

A

parainfluenza virus

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12
Q

what are 2 key features of croup caused by parainfluenza virus?

A

improves in <48 hours
responds well to dexamethasone

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13
Q

what are 3 other common pathogenic causes of croup

A

influenza
adenovirus
RSV

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14
Q

what is a possible cause of croup especially in pre/unvaxinated children?

A

diphtheria - leads to epiglottitis!!! high mortality!

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15
Q

what are 5 symptoms of croup?

A

increased work of breathing
barking cough in clusters
hoarse voice
stridor
low grade fever

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16
Q

what is the treatment for croup?

A

supportive

oral dexamethasone - single dose 150 mcg/Kg (can repeat at 12 hours)

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17
Q

what is the stepwise management of severe croup?

A

oral dexamethasone
O2
Neb budesonide
Neb adrenaline
Intubation and ventilation

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18
Q

what age is virally induced wheeze typical in?

A

<3 years

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19
Q

what is the pathophysiology of viral induced wheeze?

A

viral infection causes inflammation and oedema which reduces space for air flow in a greater proportion due to small size of child’s airways- Poiseuilles law

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20
Q

what is the management of viral induced wheeze?

A

same as acute asthma

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21
Q

what kind of wheeze is heard in asthma and viral wheeze?

A

expiratory wheeze throughout

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22
Q

what is moderate asthma?

A

peak flow >50% predicted
normal speech
otherwise well

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23
Q

what is severe asthma? (6)

A

peak flow <50% predicted
sats <92%
unable to complete sentences
signs of respiratory distress
RR >40 1-5years, >30 5+ years
HR >140 1-5 years, >125 5+ years

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24
Q

what is life threatening asthma?

A

peak flow <33% predicted
sats <92%
exhaustion and poor resp effort
hypotension
silent chest
cyanosis
altered consciousness/confusion

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25
what is a physical finding on the chest wall in severe chronic asthma?
harrison sulus - indentation in chest wall along 6th rib can be bilateral or unilateral
26
what is the acute management of asthma/viral wheeze?
Escalating Bronchodilators - Salbutamol, ipratropium bromide, magnesium sulphate, aminophylline PLUS - Steroids - prednisone (oral) or Hydrocortisone IV ABx - if suspected
27
what is the stepwise progression of bronchodilators in acute asthma?
IHR/Neb salbutamol (10 puffs every 2 hours or repeat nebs every 20-30 mins) IHR/Neb ipratropium bromide IV magnesium sulphate IV aminophylline
28
what on spirometry suggests an obstructive picture?
reversibility FEV1 <80%, FEV1:FVC <0.7
29
what are 4 investigations for asthma?
spirometry direct bronchial challenge fractional exhaled nitric oxide peak flow variability
30
what is the long term management of asthma over 5?
Reliever - SABA (salbutamol) Preventer 1 - ICS 2 - ICS + LTRA (leukotriene receptor antagonist - montelukast) 3 - ICS + LABA (-LTRA) 4 - MART (maintenance and reliever therapy, steroid + (S)LABA)
31
which LABA can be used as a short acting agonist also?
folmeterol
32
can ICS cause restricted growth?
can reduced adult hight with long term use by up to 1cm . dose dependent effect
33
what is the most common cause of bronchiolitis?
Respiratory syncytial virus (RSV)
34
in what age group does bronchiolitis occur?
<1 year, most common <6 months can be up to 2 years
35
what are 8 typical signs of bronchiolitis?
coryzal symptoms - URTI signs of resp distress dyspnoea tachypnoea poor feeding mild fever apnoea wheeze and crackles
36
what are 8 signs of respiratory distress?
raised RR use of accessory muscles intercostal and subcostal recession nasal flaring head bobbing tracheal tugging cyanosis abnormal airway noise
37
what is the typical course of RSV bronchiolitis?
coryzal symptoms chest symptoms 1-2 days later worst day 3-4 symptoms usually last 7-10 days
38
what are reasons for admission in bronchiolitis?
<3 months, pre-existing conditions <50-70% of normal milk intake clinical dehydration RR >70 O2 <92% resp distress apnoea parent not able to manage/access medical help at home
39
what is the management of bronchiolitis?
ensure adequate intake - NG, IVs saline nasal drops, nasal suctioning O2 ventilation support
40
2 signs of poor ventilation of a cap gas?
rising pCO2 falling pH
41
what can be given as prevention of RSV infection?
Palivizumab monthly injection as prevention of bronchiolitis caused by RSV given to high risk babies. Provides passive protection
42
what age does laryngomalacia occur at?
infants peaking at 6 months - inspiratory stridor exacerbated by lying, feeding, upset => no coinciding resp distress problem resolves as larynx matures - around 1.5 years
43
what chromosome is CF carried on?
chromosome 7
44
mutations on what gene causes CF?
Cystic fibrosis transmembrane conductance regulatory gene
45
what channels are affected in CF?
chloride channels
46
what is often the first sign of CF?
meconium ileus - not passing meconium in 24 hours, abdo distention, vomiting
47
what are 7 causes of clubbing in children?
hereditary cyanotic heart disease infective endocarditis CF TB IBD Liver cirhosis
48
what is the diagnostic chloride concentration on sweat test for CF?
>60 mmol/L
49
which are 2 bacteria which are especially difficult to treat in CF?
pseudomonas aeruginosa Burkholderia cepacia
50
what Abx can be used to treat pseudomonas?
tobramycin nebs or oral ciprofloxacin
51
what is the management of CF?
multidisciplinary chest physio exercise high calorie diet CREON tablets - for pancreatic insufficiency prophylactic flucloxacillin Tx chest infections bronchodilators DNase nebs - help clear secretions hypertonic saline nebs vaccinations
52
what is the average life expectancy for CF?
47 years
53
what infection typically causes epiglottitis?
HiB
54
what are 8 symptoms of epiglottitis?
sore throat/pain swallowing stridor drooling tripod position high fever muffled voice scared and quiet septic and unwell
55
what investigation can be done in suspected epiglottitis?
lateral xray of neck
56
what is the management of epiglottitis?
alert senior paediatrician and anaesthetist ABx - cefriaxone steroids - dexamethasone
57
what is a complication of epiglottitis?
epiglottic abscess
58
what is chronic lung disease of prematurity (bronchopulmonary dysplasia)?
typically <28 weeks gestation suffer from resp distress syndrome and require O2 or intubation and ventilation at birth. Diagnosed on CXR when infant required O2 >36 weeks gestational age
59
what are 5 features of chronic lung disease of prematurity?
low O2 sats increased work of breathing poor feeding and weight gain crackles and wheeze increased susceptibility to infection
60
what can reduce risk of chronic lung disease of prematurity?
corticosteroids (betamethasone) in premature labour mothers <36 weeks CPAP rather than intubation Caffeine to stimulate resp effort avoid over-oxygenating
61
what is the most common pathogen of otitis media?
strep pneumoniae
62
what is the typical presentation of otitis media?
ear pain reduced hearing symptoms of URTI
63
what is the first line Abx for otitis media?
1 - amoxicillin 5 days erythro, clarithro
64
what is glue ear?
otitis media with effusions - middle ear become full of fluid due to a blockage in the eustacion tube causing loss of hearing
65
what can be seen on otoscopy in glue ear?
dull tympanic membrane with air bubbles or a visible fluid level can look normal
66
what is the natural course of glue ear?
usually resolves within 3 months without treatment
67
how long does it usually take grommets to fall out?
1 year
68
what are 3 common congenital causes of deafness?
maternal rubella or CMV genetic deafness associated syndromes - downs
69
what are 2 common perinatal causes of deafness?
prematurity hypoxia during or after birth
70
what are 4 common post natal causes of deafness?
jaundice meningitis and encephalitis otitis media or glue ear chemo
71
what is the range of normal hearing on an audiogram?
all readings between 0-20 dB
72
what are 3 symptoms additional to tonsillitis symptoms that can indicate quinsy?
trismus - unable to open mouth change in voice (hot potato voice) swelling and erythema surrounding tonsils
73
what is the most common pathogen in quinsy?
strep pyogenes (A) and Haemophilus influenzae
74
what is the management of quinsy?
drainage and Abx (co-amox) ?dex
75
what is preorbital cellulitis?
infection of upper and lower eyelid which causes red how skin swelling around eyelid and eye - distinguish from orbital cellulitis with CT scan usually no pain or reduction in eye movements, no change in vision or abnormal pupillary response
76
what is the management of preorbital cellulitis?
systemic Abx
77
what is squint also known as?
strabismus - misalignment of the eyes causing double vision
78
what are 5 causes of squint?
idiopathic hydrocephalus cerebral palsy space occupying lesion trauma
79
what are 2 tests for squint?
cover test herschberg's test
80
what is the management of squint?
occlusive patch or atropine drops in good eye
81
what are the 3 foetal circulation shunts?
ductus venosus foramen ovalae ductus arteriosus
82
what is the ductus venosus?
shunt connecting umbilical vein to inferior vena cava allowing foetal blood to bypass liver
83
what is the foramen ovale?
shunt connecting R to L atrium which allows blood to bypass R ventricle and pulmonary circulation
84
what is the ductus arteriosus?
shunt connecting pulmonary artery and aorta which allows blood to bypass the pulmonary circulation
85
are atrial septal defects cyanotic?
no - unless R sided pressure becomes so great due to pulmonary hypertension the shunt reverses - EISENMENGER SYNDROME
86
what is eisenmenger syndrome?
when a shunt reverses due to pulmonary HTN causing R sided pressure > L sided pressure leading to pulmonary circulation bypass and cyanosis. develops after 1-2 years with large shunts and in adulthood with small
87
what are 4 complications of atrial septal defects?
stroke - with DVT - clot bypasses lungs AF or atrial flutter Pulmonary hypertension and r sided HF Eismenger syndrome
88
what are 3 types of atrial septal defect?
Ostium secondum patent foramen ovale ostium primum - tend to causes Av valve defects also
89
what is the murmur in ASD?
mid systolic crescendo-decrescendo murmur loudest at upper left sternal border with a fixed split second heart sound
90
what are 5 typical ASD symptoms?
SOB difficulty feeding poor eight gain LRTIs complications - heart failure, stroke
91
what can be seen on examination in eisenmengers syndrome?
cyanosis clubbing dyspnoea plethoric complection (due to polycythaemia)
92
what is the management of eisenmengers syndrome?
Heart lung transplant Management of pulmonary HTN, polycythaemia and thrombosis
93
what genetic conditions are associated with VSDs?
Downs and turners
94
what 3 defects can cause eisenmenger's syndrome?
ASD VSD patent duct arteriosus
95
what is the murmur is VSD?
pan-systolic more prominent at left lower sternal border in 3/4 intercostal space. May be systolic thrill
96
what are the 3 causes of pan-systolic murmur?
VSD mitral regurg tricuspid regurg
97
how long does it usually take for the ductus arteriosus to close?
1-3 days to stop functioning, 2-3 weeks to fully close
98
what kind of shunt is patent duct arteriosus?
left to right - from aorta to pulmonary vessels
99
what murmur is heard in patent duct arteriosus?
continuous crescendo -decrescendo machinery murmur
100
what causes an ejection systolic murmur? (3)
aortic stenosis pulmonary stenosis hypertrophic obstructive cardiomyopathy
101
what 2 conditions that cause cyanotic heart disease?
transposition of the great arteries Tetralogy of fallot Eisenmengers transformation
102
what condition is coarctation of the aorta associated with?
turners syndrome
103
what is the presentation of coarctation of the aorta?
weak femoral pulses - upper limb BP higher than lower limb Systolic murmur below left clavicle tachypnoea poor feeding grey, floppy baby
104
what is the management of coarctation of the aorta?
surgery in high risk - prostaglandin E to keep ductus arteriosus open till surgery
105
what cardiac condition causes cyanosis at birth?
transposition of the great arteries
106
what is the management of transposition of the great arteries?
prostaglandins - maintain ductus arteriosus Balloon septostomy - insert balloon catheter into foramen ovale to create largeASD open heart surgery - arterial switch
107
what are the 4 pathologies in tetralogy of fallot?
Pulmonary valve stenosis Right ventricular hypertrophy Overriding Aorta VSD PROVe
108
what kind of shunt is there in tetralogy of fallot?
left to right - cyanotic
109
what are 4 risk factors for tetralogy of fallot?
rubella infection increased age of mother alcohol in pregnancy diabetic mother
110
what investigations can be used for septal defects?
echo + doppler flow studies
111
what can be seen on chest x-ray in tetralogy of fallot?
boot shaped heart due to right ventricular hypertrophy
112
what are 6 manifestations of tetralogy of fallot?
cyanosis clubbing poor feeding poor weight gain ejection systolic murmur loudest in pulmonary area tet spells
113
what are tet spells?
seen in tetralogy of fallot where shunt becomes temporarily worse causing cyanotic episode - usually during waking, exertion, crying
114
what is the management of tet spells?
squatting or pulling knees to chest - increases systemic vascular resistance O2 beta blockers - relax R ventricle IV fluids morphine - decrease resp drive sodium bicarb - buffer metabolic acidosis phenylephrine infusion - increase systemic vascular resistance
115
what is the management of tetralogy of fallot?
Prostaglandins - maintain ductus arteriosus surgery
116
what is ebstein's anomaly?
R tricuspid valve lower than normal causing R ventricle to be smaller causing poor flow to pulmonary vessels - usually presents after closing of ductus arteriosus
117
what 4 conditions are associated with pulmonary valve stenosis?
tetralogy of fallot william syndrome noonan syndrome and turners syndrome congenital rubella syndrome
118
what is rheumatic fever?
autoimmune condition triggered by antibodies to group A beta-haemolytic strep - typically strep pyogenes causing tonsilitis. The process usually occurs 2-4 weeks after initial infection
119
what is the presentation of rheumatic fever? (7)
fever joint pain - migratory of large joints erythema marginatum rash shortness of breath chorea firm painless nodules carditis - tachy/brady, murmurs, pericardial rub, heart failure
120
what are 3 investigations for rheumatic fever?
throat swab and culture Anti streptococcal antibodies (ASO) titres ECHO, ECG, CXR
121
what criteria is used to diagnose rheumatic fever?
jones criteria
122
what is the jones criteria?
for diagnosing rheumatic fever TWO of JONES Joint arthritis Organ inflammation (carditis) Nodules Erythema marginatum rash Sydenham chorea OR One JONES and TWO FEAR Fever ECG changes (prolonged PR) Arthralgia Raised inflammatory markers
123
what is the management of rheumatic fever?
Abx - Benzathine benzylpenicilin or phenoxymethylpenicillin NSAIDs or salicates (joint pain) Aspirin and steroids - carditis Heartfailure - Diuretics +/- ACEi Chorea - carbamazepine
124
what are 3 complications of rheumatic fever?
recurrence valvular heart disease - mitral stenosis chronic heart failure
125
what are 6 signs of problematic reflux in babies?
chronic cough hoarse cry distress after feeding reluctance to feed pneumonia poor weight gain
126
what advice can be given for GORD in babies?
small frequent meals burping regularly not overfeeding keep baby upright after feeding
127
what kind of vomiting is present in GORD?
effortless mainly after feeding
128
what 3 treatments can be given in babies with GORD?
gaviscon mixed with feeds thickened milk or anti-reflux formula PPIs
129
what is a key feature of pyloric stenosis?
projectile vomiting
130
when does pyloric stenosis present?
first few weeks (4-6 weeks) of life baby failing to thrive with projectile vomiting
131
what 3 things can be found on examination of a baby with pyloric stenosis?
observation of stomach peristalsis firm round 'olive like' mass in upper abdomen hypochloric (low Cl-) metabolic alkalosis on blood gas
132
how is pyloric stenosis dianosed?
Abdo USS
133
what is the management of pyloric stenosis?
laparoscopic pyloromyotomy - Ramstedt's operation
134
what are 2 common viral causes of gastroenteritits?
rotavirus norovirus
135
which e.coli produces shiga toxin?
E.coli 0157
136
what does shiga toxin cause?
abdo cramps, bloody diarrhoea, vomiting - can lead to haemolytic uraemic syndrome
137
what is the most common cause of bacterial gastroenteritis?
campylobacter jejuni
138
where does campylobacter come from?
raw/improperly cooked poultry untreated water unpasteurised milk
139
what is the treatment of campylobacter?
azithromycin or ciprofloxacin
140
which bacteria causes gastroenteritis after food has not been refrigerated quickly (rice)?
bacillus cereus - diarrhoea resolving in 24 hours
141
what is the treatment of Giardiasis?
metronidazole
142
what are 4 possible complications of gastroenteritis?
lactose intolerance IBS reactive arthritis GBS
143
what is toddlers diarrhoea?
chronic watery diarrhoea in well <5 year olds which can often be helped by changes in diet
144
what are the 4fs of toddler's diets?
fat - shouldnt have low fat diet Fluid - not sugary drinks, not too much water Fruit + fruit juice - gut cannot absorb fructose or sorbitol easily Fibre - 12-18g per day
145
what is persistent diarrhoea?
>2 weeks
146
what is chronic diarrhoea?
>4 weeks
147
what is encopresis?
foecal incontinence
148
what are 5 causes of faecal incontinence?
spina bifida hirschprungs disease cerebral palsy overflow incontenence - stress, abuse Constipation with overflow
149
what are 5 lifestyle factors that may contribute to constipation in children?
habitual not opening bowels low fibre diet poor fluid intake sedentary lifestyle psychosocial problems
150
what are 8 secondary causes of constipation in children?
hirschprungs disease CF Hypothyroid Spinal cord lesions sexual abuse Intestinal obstruction anal stenosis cow milk intolerance
151
what is the first line laxative in children?
movicol
152
when is the peak incidence of apendicitis?
10-20 years
153
where is McBurney's point?
one third of the way from the anterior superior iliac spine and the umbilicus
154
what is rovsing's sign?
for apendicitis when palpation in LIF causes pain in RIF
155
what are 6 classical features of apendicitis?
loss of appetite nausea and vomiting rovsing's sign guarding rebound tenderness percussion tenderness
156
what are 5 key differentials for apendicitis?
ectopic pregnancy ovarian cysts meckel's diverticulum mesenteric adenitis appendix mass
157
what is meckel's divertculum?
malformation of distal ileum present in 2% of population that can bleed, become inflamed, rupture or cause volvutus, intussusception or obstruction
158
what are 5 complications of apendicectomy?
bleeding, infection, pain, scars damage to bowel or bladder removal of normal appendix anaesthetic risks VTE
159
what are the 5 key features of Crohn's? (mneumonic)
NESTS No blood or mucus (less than UC) Entire GI tract Skip lesions Terminal ileum most affected + Trans mural Smoking risk factor weight loss, strictures, fistulae
160
what are the 7 key features of UC? (mneumonic)
CLOSEUP Continuous inflammation Limited to colon and rectum Only superficial mucosa Smoking protective Excrete blood and mucus Use aminosalicylates Primary sclerosing cholangitis
161
what are extra-intestinal manifetations of IBD?
finger clubbing erythema nodosum pyoderma gangrenosum episcleritis and iritis inflammatory arthritis primary sclerosing cholangitis (UC)
162
what is one stool test that can be used for IBD but only in adults?
faecal calprotectin
163
what is the acute management of crohn's?
1 - steroids (oral pred or IV hydrocortisone) + immunosuppression under specialist - azathioprine, mercaptopurine, methotrexate, infliximab
164
what is the long term management of crohn's?
1 - azithioprine or mercaptopurine
165
what is the management of mild-moderate acute UC?
1 - aminosalcylates - mesalazine 2 - corticosteroids
166
what is the management of severe acute UC?
1 - IV corticosteroids (hydrocortisone) 2 - IV ciclosporin
167
what is the long term management of UC?
aminosalicylate - mesalazine or azathioprine or meraptopurine
168
what is the name of surgery to remove the colon and rectum sometimes used in UC?
panproctocolectomy
169
what is the most common congenital abnormality of the small bowel?
meckel's diverticulum
170
when do people usually present with meckel's diverticulum?
<2 years
171
what are 2 antibodies in coeliac disease?
anti-tissue transglutaminase (anti-TTG) anti-endomysial (anti-EMA)
172
What skin manifestation can be a sign of coeliac disease?
dermatitis herpetiformis - itchy blistering skin rash usually on abdomen
173
what are 3 neurological signs associated with celiac disease?
peripheral neuropathy cerebellar ataxia epilepsy
174
what are 2 findings on endoscopy in coeliac disease?
crypt hypertrophy villous atrophy
175
what are 6 conditions associated with coeliac?
T1DM thyroid disease autoimmune hepatitis primary billiary cirrhosis primary sclerosing cholangitis downs syndrome
176
what are 7 complications of untreated coeliac disease?
vitamin deficiency anaemia osteoporosis ulcerative jenunitits enteropathy associated t-cell lymphoma non-hodgekin lymphoma small bowl adenocarcinoma
177
what is faltering growth?
fall in weight across: 1+ centiles if birth weight below 9th 2+ centiles if birth weight between 9th-91st 3+ centiles if birth weight 91+ centiles
178
what are 5 causes of failure to thrive?
inadequate nutritional intake difficulty feeding malabsorption increased energy requirements inability to process nutrition
179
what are 5 causes of inadequate nutritional intake in children failing to thrive?
maternal malabsorption if breastfeeding IDA family or parental problems neglect availability of food
180
what are 4 causes of difficulty feeding?
poor suck (cerebral palsy) cleft lip/palate genetic conditions w/ abnormal face structure pyloric stenosis
181
what are 5 causes of malabsorption in children failing to thrive?
CF coeliac disease cows milk intolerance chronic diarrhoea IBD
182
what are 4 causes of increased energy requirements in children failing to thrive?
Hyperthroidism chronic disease - CF, CHD malignancy chronic infections - HIV, immunodeficiency
183
what are 2 causes of inability to process nutrients in children failing to thrive?
inborn errors of metabolism T2DM
184
how do you calculate mid-parental height?
average of parents height
185
what is anthropometry?
in nutritional assessment - includes weight, height mid-upper arm circumference and skinfold thickness
186
what is marasmus?
severe malnutrition due to inadequate energy intake in all forms. More common <1 year olds and causes muscle wasting and loss of subcutaneous fat but no oedema
187
what is kwashiorkor?
Severe malnutrition due to protein deficiency but with adequate energy intake. Leads to oedema, ascites and enlarged liver with fatty infiltrates
188
what is Hirschprungs disease?
congenital condition where nerve cells of the myenteric (auerbach's) plexus are absent causing a lack of peristalsis of the large bowel
189
what is the key pathopysiology of hirschprungs disease?
absence of parasympathetic ganglion cells in the colon
190
what are 4 syndrome that can be associated with hirschprung's?
downs neurofibromatosis waardenburg syndrome multiple endocrine neoplasia type II
191
what are 5 presentations of hirschprungs disease?
delay in passing meconium chronic constipation since birth abdo pain and distention vomiting poor weight gain and failure to thrive
192
what is hirschsprung associated enterocolitis?
inflammation and obstruction of the intestines in 20% of neonates with hirschprungs presenting usually between 2-4 weeks with fever, abdo distension, diarrhoea (w/ blood usually) and features of sepsis
193
what is the management of hirschsprungs associated enterocolitis?
Abx fluid resuscitation decompression of obstruction
194
how is hirschsprungs disease diagnosed?
rectal biopsy + histology for absence of ganglionic cells
195
what is the management of hirschsprungs disease?
surgical resection of aganglionic bowel
196
what age group does intussusception occur most commonly in?
6 months - 2 years more common in boys
197
what is intussusception?
when the bowel invaginates into itself causing bowel obstruction
198
what 5 things are risk factors for intussusception?
concurrant viral illness henoch-schonlein purpura CF intestinal polyps meckel diverticulum
199
what are 6 presentations of intussusception?
sever colicky abdo pain pale, lethargic unwell child redcurrant jelly stool sausage shaped RUQ mass vomiting intestinal obstruction
200
what is the first line investigation for intussusception?
USS
201
what is the treatment for intussusception?
1 - therapeutic enema - contrast, water or air 2- surgery
202
what are 4 complications of intussusception?
obstruction gangrenous bowel perforation death
203
what are 8 causes of bowel obstruction in children?
meconium ileus hirschprungs oesophageal atresia duodenal atresia intussusception imperforate anus malrotation of the intestines + volvulus strangulated hernia
204
what can be seen on abdo xray in obstruction?
proximal dilated bowel loops distal collapsed bowel loops absence of air in rectum
205
when does biliary atresia present?
shortly after birth
206
what kind of bilirubin is high in biliary atresia?
conjugated bilirubin
207
what is the 1st line investigation for billiary atresia?
conjugated and unconjugated bilirubin
208
what is classed as persistent jaundice in babies?
>14 days term babies >21 days premies
209
what is the management of billiary atresia?
surgery - kasai portoenterostomy often require full liver transplant
210
what is henoch-schonlein purpura?
autoimmune condition usually triggered by URTI which causes inflammation of capilaries leading to purpuric rash typically on lower legs, joint pain and GI symptoms. Can also cause kidney damage
211
what are the 3 key features of Henoch-schonlein purpura?
purpuric rash on legs joint pain GI symptoms
212
what is an abdominal migraine?
severe central abdo pain >1 hour with normal examination may also have nausea, vom, anorexia, pallor, headache, photophobia, aura
213
what is the acute management of abdo migraine?
paracetamol ibuprofen sumatriptan
214
what is the prophylaxis of abdo migraine?
1 - pizotifen - serotonin agonist propanolol cyproheptadine - antihistamine flunarazine - Ca channel blocker
215
what is gastroschisis?
a birth defect causing abdominal organs to be located outside abdomen due to defect in abdominal wall
216
what are 2 examination findings in diaphragmatic hernia?
apex beat displaced to r side of chest poor air entry in L chest
217
what is the management of diaphragmatic hernia?
NG tube suction to prevent intrathoracic bowel distention and surgical repair - high mortality due to underdeveloped lungs in utero
218
who are umbilical hernias more common in?
children of african descent
219
what are 2 classes of symptoms of cow milk protein allergy?
GI symptoms - bloating, wind, abdo pain, diarrhoea , vomiting Allergic symptoms - hives, angio-oedema, cough/wheeze, sneezing, watery eyes, eczema, anaphylaxis
220
what formula can be used in cow milk protein allergy?
hydrolysed formulas or in severe cases elemental amino acid formulas
221
what is the difference between cow milk intolerance and allergy?
intolerance has GI symptoms but no allergic symptoms unlike allergy
222
what immunoglobulin causes rapid cows milk protein allergy?
IgE
223
what are choledochal cysts?
congenital cyst dilations of the billary tree more commonly affecting females which have a small risk of malignancy
224
what is the classic triad of choledochal cysts?
abdo pain jaundice abdo mass
225
what is neonatal hepatitis syndrome?
idiopathic prolonged neonatal jaundice and hepatic inflammation which may cause low birth weight and faltering growth
226
what is the usual presentation of colic?
sudden inconsolable crying/screaming accompanied by drawing knees to chest and passign excessive gas
227
what 2 findings indicate pyelonephritis over uti?
temp >38 degrees loin pain/tenderness
228
what is the management for < 3 months with fever?
immediate IV Abx - cefotaxime+ amoxicillin + full septic screen
229
what a full septic screen in <3 months?
bloods - FBC, CRP, cultures, lactate urine dip CXR LP ? cap gas
230
when should children with UTIs get an USS within 6 weeks?
if first UTI <6 months recurrent UTIs atypical utis
231
what investigation can be done for defects in renal tissue and scars after UTI?
DMSA scan (dimercaptosuccinic acid) - injection of radioactive material using gamma camera done 4-6 months after infection
232
what investigation can be done to visualise urinary anatomy, vesicouteric reflux and urethral obstruction in children <6 months?
micturating cystourethrogram (MCUG) - catheterise child and inject contrast then take x rays
233
what is vesico-uteric reflux?
where there is a developmental abnormality of the vesicouteric junction where the ureters are displaced laterally and enter the bladder directly allowing urine to reflux back up ureters and to the kidney causing dilatation and increased risk of infection and scaring
234
what is the management of pyelonephritis in children >3 months?
oral trimethoprim 7 days or IV co-amox 2-4 days
235
what is the management of UTI in children >3 months?
oral ABx - trimeth or nitro 3 days
236
what is enuresis?
involuntary urination
237
by what age do children usually get control of day time bladder function?
2 years
238
by what age do children usually stop bed wetting?
3-4 years
239
what are 5 possible causes of primary nocurnal enuresis?
overactive bladder - frequent small volume urination prevents development of bladder capacity fluid intake prior to bed failure to wake psychological distress secondary causes - chronic constipation, UTI, learning disability, cerebral palsy
240
what is primary nocturnal enuresis?
wetting the bed having never stopped
241
what is secodnary nocturnal enuresis?
wetting the bed having previously stopped for >6 months
242
what are 5 causes of secondary nocturnal enuresis?
UTI constipation T1DM psychosocial problems abuse
243
what is diurnal enuresis?
day time wetting self
244
what are 3 management options for nocturnal enuresis?
Eneurisis alarm - 1st line desmopressin (taken at bedtime) oxybutinin - anticholinergic imipramine - tricyclic antidepressant
245
what age range is nephrotic syndrome most common in?
2-5 years
246
what is the classical triad of nephrotic syndrome?
low serum albumin high urine protein (3+ or >3 grams) oedema
247
what are 6 signs of nephrotic syndrome?
low serum albumin high urine protein oedema deranged lipid profile high BP hyper-coagulability
248
what is the most common cause of nephrotic syndrome in children?
minimal change disease
249
what are 4 secondary causes of nephrotic syndromes?
intrinsic kidney disease - focal segmental glomerulosclerosis, mebranoproliferative glomerulonephitis Henoch schonlein purpura diabetes infection - HIV, hepatitis, malaria
250
what can be seen on urinalysis in minimal change?
small molecular weight proteins and hyaline casts
251
what is the first line management of minimal change disease?
high dose corticosteroids - prednisolone
252
what is the management of steroid resistant nephrotic syndrome?
ACEi immunosupressants - cyclosporine, tacrolimus, rituximab
253
what is the general management of nephrotic syndromes (5)?
high dose steroids - pred low salt diet diuretics albumin infusion antibiotic prophylaxis
254
what are 5 complications of nephrotic syndromes?
hypovolaemia and low BP thrombosis infection - kidneys leak immunoglobulins acute or chronic renal failure relapse
255
what is the pathophysiology of nephrotic syndromes?
the basement membrane of the glomerulus becomes highly permeable to proteins allowing them to leak from the blood into the urine
256
what is the pathophysiology of nephritic syndromes?
inflammation of the nephrons
257
what are 3 consequences of nephritic syndromes?
reduction in kidney function haematuria proteinuria (less than nephrotic)
258
what are the two most common causes of nephritis in children?
post-streptococcal glomerulonephritis IgA nephropathy
259
what is the pathophysiology of post-streptococcal glomerulonephritis?
1-3 weeks after b-haemolytic strep (strep pyogenes) infection e.g tonsilitis immune complexes get stuck in the glomeruli and cause inflammation leading to AKI
260
what 2 investigations can be done to test for strep in post-strep glomerulonephritis?
positive throat swab anti-streptolysin antibody titres
261
what is the management of nephritis?
supportive mainly manage complications - IgA may need immunosupression
262
what is IgA nephropathy a complication of?
Henoch-schonlein purpura (IgA vasculitis)
263
what is the pathophysiology of IgA nephropathy?
IgA deposits in nephrons cause inflammation
264
what 2 things can be seen on biopsy in IgA nephropathy?
IgA deposits Glomerular mesangial proliferation
265
what age group is usually affected with IgA nephropathy?
teens and young adults
266
what usually triggers haemolytic uraemic syndrome?
shinga toxin (from e.coli 0157 or shingella)
267
what is the classical triad of haemolytic uraemic syndrome?
haemolytic anaemia AKI thrombocytopenia
268
what increases the risk of haemolytic uraemic syndrome?
use of antibiotics and loperamide to treat gastroenteritis caused by e.coli0157 or shingella
269
how long after onset of gastroenteritis do symptoms of haemolytic uraemic syndrome usually start?
5 days
270
what are 8 presentations of haemolytic uraemic syndrome?
reduced urine output haematuria or dark brown urine abdo pain lethargy and irritablility confusion oedema HTN bruising
271
what is the management of haemolytic uraemic syndrome?
supportive - renal dialysis, antihypertensives, maintain fluid balance, blood transfusion
272
what is hypospadias?
condition where uretheral meatus is displaced to underside of penis
273
what is epispadias?
condition where the urethral meatus is displaced to the top side of the penis
274
what is chordee?
where head of penis is bent downwards
275
what are 3 complications of hypospadias?
difficulty directing urination cosmetic and psychological concerns sexual dysfunction
276
what is phemosis?
pathological non-retration of foreskin
277
what condition is the most common cause of phimosis?
balantitis xerotica obliterans
278
what reduction in renal function classes as AKI?
<0.5 ml/Kg/hour over 6 hours
279
what category of cause is most common in childhood AKI?
pre-renal
280
what are the 2 most common causes of intra-renal failure in children?
haemolytic uraemic syndrome acute tubular necrosis
281
what is stage 1 ckd?
eGFR >90 ml/min per 1.73 m2
282
what is stage 2 ckd?
eGFR 60-89
283
what is stage 3 ckd?
eGFR 30-59
284
what is stage 4 eGFR?
eGFR 15-29
285
what is stage 5 ckd?
eGGR <15 ml/min per 1.73m2
286
what 8 presenting features of severe CKD?
anorexia and lethargy polydipsia and polyuria faltering growth bony deformities from renal rickets hypertension acute on chronic renal failure proteinuria normochomic normocytic anaemia
287
what type of hypersensitivity reaction in anaphylaxis?
type 1
288
what immunoglobulin causes anaphylaxis?
IgE
289
what is the pathophysiology of anaphylaxis?
igE stimulates mast cells to rapidly release histamine in mast cell degranulation
290
what is the key feature of anaphylaxis vs non-anaphylactic allergy?
anaphylaxis causes compromise of airway, breathing or circulation
291
what are the 4 allergic symptoms?
urticaria itching angio-oedema abdominal pain
292
what is the management of anaphylaxis?
ABCDE IM adrenaline Antihistamines - chlorphenamine or certirizine steroids - hydrocortisone
293
what can be a complication of anaphylaxis?
biphasic reaction - second anaphylactic reaction after treatment
294
what investigation can be done for anaphylaxis?
serum mast cell tryptase - within 6 hours of event
295
what are 5 reasons someone with a non-anaphylactic allergic reaction may get an epipen?
asthma requiring ICS poor access to medical tx adolescents - higher risk nut/sting allergies significant co-morbidities
296
what is the skin sensitisation theory of allergy?
there is a break in the infants skin that allows allergens from the environment to react with the immune system but there is no contact with the allergen through the GI tract
297
what is the classification system for hypersensitivity reactions?
cooms and gell
298
what is a type 1 hypersensitivity reaction?
IgE antibodies trigger mast cells and basophils to degranulate causing an immediate reaction - food allergy reactions
299
what is a type 2 hypersensitivity reaction?
IgG and IgM mediated activating the complement system leading to direct damage of local cells - haemolytic disease of newborn, transfusion reactions
300
what is a type 3 hypersensitivity reaction?
immune complexes accumulate and cause damage to local tissues - autoimmune conditions
301
what is a type 4 hypersensitivity reaction?
cell mediated caused by T lymphocytes which are inappropriately activated causing inflammation and damage to local tissues - organ transplant rejection, contact dermatitis
302
what are 3 investigations for allergies?
skin prick test RAST testing - measure of allergen specific IgE food challenge - gold standard
303
what hypersensitivity reaction is allergic rhinits?
type 1 IgE
304
what is the presentation of allergic rhinitis?
runny, bocked, itchy nose sneezing itchy, red, swollen eyes
305
what are 3 non-sedating antihistamines?
certirizine loratadine fexofenadine
306
what are 2 sedating anti-histamines?
chlorpenamine promethazine
307
what can be taken as prophylactic for allergic rhinits?
nasal corticosteroids - fluticasone, mometasone
308
what are 3 live vaccinations?
BCG MMR nasal flu vaccine
309
where is eczema usually found?
flexor surfaces
310
what are 2 thick emolients?
50;50 ointment hydromol
311
what is the steroid cream ladder?
mild - hydrocortisone 0.5,1,2.5% moderate - eumovate 0.05% potent - betamethasone 0.1% very potent - clobetasol propionate 0.05%
312
what is the most common bacterial infection in eczema?
s aureus
313
what abx can be used to treat bacterial infection of eczema?
flucloxacillin
314
what is eczema herpeticum?
viral skin infection caused by HSV-1 or varicella zoster
315
what is the presentation of eczema herpeticum?
eczema sufferer developed widespread painful vesicular rash with fever, lethargy, irritability, reduced oral intake and swollen lymph nodes
316
What is the management of eczema herpeticum?
aciclovir
317
what is stevens-johnson syndrome and toxic epidermal necrosis?
a disproportionate immune response causing epidermal necrosis resulting in blistering and shedding of top layer of skin - SJS <10% of body, TEN >10% of body
318
what are 5 medications that can cause stevens-johnson syndrome?
anti-epileptics(lamotrigine, phenotoin) antibiotics allopurinol NSAIDs Penicillin
319
what are 7 infections that can cause stevens-johnson syndrome?
herpes HIV Mumps FLu EBV mycoplasma pneumonia CMV
320
what is the natural history of stevens-johnson syndrome?
starts with fever, cough, sore throat, mouth, eyes and skin then develop purple/red rash which blisters the after a few days skin sheds leaving raw tissue underneath can also affect internal organs
321
what is the management of stevens-johnson syndrome?
admit to derm/burns unit steroids immunoglobulins immunosuppression
322
what are 3 complications of stevens-johnson syndrome?
secondary infection permanent skin damage and scarring visual complications with eye involvement
323
what are 6 triggers for acute urticaria?
allergies contact with chemicals, latex or nettles medications viral infection insect bites rubbing skin
324
what are the 3 different types of chronic urticaria?
chronic idiopathic urticaria chronic inducible urticaria autoimmune urticaria
325
what are 6 triggers for chronic inducible urticaria?
sunlight temperature change exercise strong emotions hot/cold weather pressure
326
what is the management of urticaria?
Antihistamines - fexofenadine if problematic - anti-leukotrienes (montelukast), omalizumab, cyclosporin
327
what can cafe-au-lait spots be a sign of?
neurofibromatosis type 1 (if have 6+)
328
what 3 things can port wine marks rarely be a sign of?
sturge-weber syndrome klippel trenaunay syndrome macrocephaly-capillary malformation
329
what are mongolial blue spots?
blue/black macular discolouration at base of spine and on buttocks or thighs - fade over first few years of life
330
what is osteogenesis imperfecta?
genetic condition also known as brittle bone disease caused by mutation in formation of collagen
331
what are 8 features associated with osteogenesis imperfecta?
hypermobility blue/grey sclera triangular face short stature deafness from early adulthood dental problems bone deformities joint and bone pain
332
what is the pathophysiology of rickets?
deficiency in vitamin D/calcium which results in defective bone mineralisation
333
what is the name of the genetic version of rickers?
hereditary hypophasphataemic rickets
334
what are 8 presentations of rickets?
lethargy bone pain swollen wrists bone deformuty poor growth dental problems muscle weakness pathological or abnormal fractures
335
what are 5 bone deformities in rickets?
bowing of legs knock knees rachitic rosary - expanded ribs causing lumps on chest craniotabes - soft skull, delayed closing delayed teeth
336
what investigation is done for vitamin d deficiency?
serum 25-hydroxyvitamin D - <25nmol/L = deficiency
337
what are 5 investigations that can be done for rickets?
serum 25-hydroxyvitamin D serum calcium serum phosphate serum alkaline phosphatase parathyroid hormone
338
what is the most common cause of hip pain in children 3-10 years?
transient synovitis
339
what is transient synovitis often associated with?
preceeding viral upper resp tract infection
340
what age is septic arthitis most common in?
<4 years
341
what is teh most common causative organism of septic arthritis?
staph aureus
342
what are 4 investigations of the synovial fluid in septic arthritis?
gram staining crystal microscopy culture antibiotic sensitivities
343
what is the most common causative organism of osteomyelitis?
s.aureus
344
what are 6 risk factors for osteomyelitis?
open fracture orthopaedic surgery immunocompromised sickle cell HIV TB
345
what is perthes disease?
disruption of blood flow to femoral head causing avascular necrosis of the epiphysis of the femur
346
what age group does perthes disease occur most commonly in?
5-8 years - mostly in boys
347
what is slipped upper femoral eiphysis? (SUFE)
when head of femur displaces along growth plate
348
who is SUFE more common in?
boys aged 8-15 obese children
349
how do people with SUFE prefer to hold their hip?
in external rotation and have limited movement of hip
350
what is the management of SUFE?
surgery to correct femoral head position
351
what is osgood-schlatter disease?
inflammation of the tibial tuberosity where patella ligament inserts causing anterior knee pain in adolescents - usually unilateral but can be bilateral. causes hard lump at front of knee
352
what are 3 presentations of osgood-schlatter?
visible or palpabl hard tender lump at tibial tuberosity pain in anterior knee pain exacerbated by activity, kneeling or extension of knee
353
what is a complication of osgood-schlatters?
avulsion fracture - tibial tuberosity separated from tibia
354
what is the management of osgood-schlaters?
reduction in physical activity ice NSAIDs
355
what is developmental dysplasia of the hip?
structural abnormality n developmental of foetal bones leading to instability of te hips and tendency for sublaxation or dislocation
356
what are 7 risk factors for developmental dysplasia of the hip?
FHx 1st degree relative breech presentation 36 weeks multiple pregnancy female oligohydramnios prematurity macrosomnia
357
what are 5 findings that may suggest developmental dysplasia of the hip on neonatal examination?
different leg lengths restricted hip abduction on one side significant bilateral restriction in hip abduction difference in knee level with flexed hips clunking of hips
358
what are two special test for developmental dysplasia of the hio?
ortolani test barlow test
359
what is the ortolani test?
for developmental dysplasia of the hip hips and knees flexed, hips gently abducted and pushed from the back to see if they will dislocate anteriorly
360
what is barlow test?
for developmental dysplasia of the hip hips and knees flexed and gentle pressure put on knees through femur to see if hips will dislocate posteriorly
361
what investigation can be done for developmetal dysplasia of the hips?
USS
362
what is the management for developmental dysplasia of the hips?
Pavlik harness (<6 months) for 6-8 weeks surgery if harness fails or >6 months
363
what are the 5 subtypes of juvenile idiopathic arthritis?
systemic JIA polyarticular JIA Oligoarticular JIa enthesitis related arthritis juvenile psoriatic arthritis
364
what is systemic juvenile idiopathic arthritis also known as?
Still's disease
365
what are 8 manifestations of systemic JIA?
subtle salmon pink rash high swinging fevers enlarged lymph nodes weight loss joint inflammation and pain splenomegally muscle pain pleuritis and pericarditis
366
what is are 2 key complication of systemic JIA?
macrophage activation syndrome - severe inflammatory responce causing DIC, anaemia, thrombocytopenia, bleeding, non-blanching rash chronic anterior uveitis
367
what are 4 key non-infective causes of fever in children > 5 days?
kawasaki disease still disease (systemic JIA) rheumatic fever leukaemia
368
what is polyarticular JIA?
idiopathic inflammatory arthritis in 5+ joints - equivalent of RhA in adults though most children are seronegative
369
what is oligoarticular JIA?
monoarthritis affecting <4 joints, classically associated with anterior uveitis. most common in gils under 6
370
what is enthesitis related arthritis?
paediatric seronegative spondyloarthropathies. inflammatory arthritis + enthesitis (inflammation of tendon insertion points) usually HLA-B27 +ve
371
what are 5 signs of juvenile psoriatic arthritis?
places of psoriasis nail piitting onycholysis (seperation of nail bed) dactylitis enthesitis
372
what is the management of juvenile idiopathic arthritis?
NSAIDs steroids DMARDs Biologics - TNF inhibitors, infliximab etc
373
what age group does kawasaki's usually affect?
<5 years
374
what is a key complication of kawasaki disease?
coronary artery aneurysm
375
what is the pathophysiology of kawasaki disease?
systemic medium sized vessel vasculitis?
376
what are 7 key features of kawasaki?
high (>39) fever >5 days widespread erythematous maculopapular rash on trunk Oedema of hands and feed preceeding desquamation Strawberry tongue and cracked lips cervical lymphadenopathy bilateral conjunctivitis arthritis
377
what are 5 investigations for kawasaki?
FBC - anaemia, leukocytosis, thrombocytosis LFTS - hypoalbuminaemia, elevated enzymes raised inflammatory markers (particularly esr) Urinaralysis - wt cells without infection ECHO
378
what happens in the acute phase of kawasakis?
most unwell for 1-2 weeks Fever, rash, lymphadenopathy
379
what happens in the subacute phase of kawasakis?
weeks 2-4 acute symptoms settle desquamation, strawberry tongue and arthralgia occur - there is risk of coronary artery aneurysms forming
380
what happens in the covalescent stage of kawasakis?
week 2-4 remaining symptoms settle
381
what is the management of kawasaki disease?
high dose aspirin - reduce risk of throbosis IV Ig to reduce risk of coronary artery aneurysm
382
what is Reye's syndrome
a preceeding viral infection usually treated with ASPIRIN followed by acute encephalopathy and hepatic dysfunction in children and young people
383
what follow up is needed with kawasakis?
ECG and ECHO
384
what cells produce surfactant?
type II pneumocytes
385
when does surfactant start to be produced?
between 24-34 weeks gestation
386
what is required to keep the ductus arteriosus open?
prostaglandins
387
what can extended hypoxia lead to during birth?
hypoxic-ischaemic encephalopathy => cerebral palsy
388
what are 3 issues of neonatal resuscitation?
large SA to weight ratio - cold born wet and loose heat rapidly meconium aspiration
389
what are 5 principles of neonatal resuscitation?
warm baby calculate APGAR score stimulate breathing inflation breaths chest compressions
390
what can be used to manage hypoxic ischaemic encephalopathy?
therapeutic hypothermia
391
when should you calculate the APGAR score?
1, 5 and 10 minutes during resus
392
how do you stimulate breathing in neonatal resussitation?
dry vigorously with towel place baby head in neutral check for airway obstruction and consider aspiration if gasping or unable to breathe
393
how should inflation breaths be given in neonates?
2 cycles of 5 inflation breaths if no response 30s of ventilation breaths can be used then chest compressions coordinated with ventilation breaths
394
what should be used for inflation breaths in preterm vs term babies?
preterm - air and O2 term - just air
395
when should you start chest compressions in a neonate?
if HR <60 bpm despite resus and inflation breaths
396
what is the APGAR score?
for neonatal resus Appearance (skin colour) Pulse Grimmace (to stimulation) Activity (tone) respiration
397
what is the scoring for appearance in APGAR?
blue/pale centrally = 0 blue extremities = 1 pink = 2
398
what is the scoring for pulse in APGAR?
Absent = 0 <100 = 1 >100 = 2
399
what is the scoring for grimmace in APGAR?
no response = 0 little response = 1 good response = 2
400
what is the scoring for activity in APGAR?
floppy = 0 flexed arms and legs =1 active = 2
401
what is the scoring for respiration in APGAR?
absent =0 slow/irregular = 1 strong/crying = 2
402
how long should delayed cord clamping be?
1 minute
403
under what gestation can be affected by respiratory distress syndrome?
< 32 weeks
404
what xray changes are seen in respiratory distress syndrome?
ground glass appearance
405
what are 4 types of ventilatory support that may be needed by premature neonates?
intubation and ventilation endotracheal surfactant CPAP supplementary oxygen
406
what are 6 short term complications of respiratory distress syndrome?
penumothorax infection apnoea intraventricular haemorrhage pulmonary haemorrhage necrotising enterocolitis
407
what are 3 long term complications of respiratory distress syndrome?
chronic lung disease of prematurity retinopathy of prematurity neurological, hearing or visual impairment
408
what are 4 causes of hypoxic ischaemic encephalopathy?
maternal sock intrapartum haemorrhage prolapsed cord nucal cord
409
what is the staging for hypoxic ischaemic encephalopathy called?
sarnat staging
410
what is the presentation of mild hypoxic ischaemic encephalopathy?
poor feeding, generally irritable, hyper-alert resolves within 24 hours normal prognosis
411
what is moderate hypoxic ischaemic encephalopathy?
poor feeding, lethargy, hypotonic, seizures can take weeks to resolve 40% develop cerebral palsy
412
what is severe hypoxic ischaemic encephalopathy?
reduced consciousness, apnoea, flaccid, reduced or absent reflexes 50% mortality 90% develop cerebral palsy
413
what temperature is therapeutic hypothermia cooled to and for how long?
33-34 degrees for 72 hours
414
when can physiological jaundice be present?
from 2-10 days of age
415
what are 8 causes of increased production of bilirubin in neonates?
haemolytic disease of newborn ABO incompatibility Haemorrhage intraventricular haemorrhage cephalo-haemorrhage polycythaemia sepsis and DIC G6PD deficiency
416
what are 6 causes of decreased bilirubin clearance in neonates?
prematurity breast milk jaundice neonatal cholestasis extrahepatic biliary atresia endocrine disorders gilbert syndrome
417
when is jaundice always pathological?
in first 24 hours of life can be sign of sepsis
418
what is kernicterus?
brain damage due to high unconjugated bilirubin
419
what causes haemolytic disease of the newborn?
rhesus incompatability
420
what is prolonged neonatal jaundice?
14+ days in full term babies 21+ days in premature babies
421
what test is done for autoimmune haemolysis?
direct coombs test
422
what is used to monitor neonatal jaundice?
treatment threshold charts
423
what is the usual treatment of neonatal jaundice?
phototherapy with blue light (450nm)
424
what are 3 complications of kernictus?
cerebral palsy learning disability deafness
425
what is the medical management of supraventricular tachycardia?
adenosine - after valsalva manoeuvres
426
what is necrotising enterocolitis?
disorder affecting premature neonates where part of bowel becomes necrotic and can lead to perforation
427
what are 5 risk factors for necrotising enterocilitis?
very low birth weight or v premature formula feeds resp distress and assisted ventilation sepsis patent ductus arteriosus and congenital heart disease
428
what are 6 features of necrotising enterocolitis?
intolerance to feeds vomiting green bile distended tender abdomen blood in stool absent bowel sounds generally unwell
429
what investigation is good for diagnosing necrotising enterocolitis?
abdo Xray - supine and lateral
430
what can be seen on xray with necrotising enterocolitis?
dilated bowel loops bowel wall oedema pneumatosis intestinalis - gas in bowel wall pneumoperitoneum - free gas in peritoneum gas in portal veins
431
what is the management of necrotising enterocolitis?
nil by mouth iv fluids total parenteral nutrition antibiotics NG tube - to drain fluid and gas SURGERY
432
what are 8 complications of necrotising enterocolitis?
perforation and peritonitis sepsis death strictures abcess formation recurrence long term stoma short bowel syndrome
433
what are the torch congenital infections?
Toxoplasmosis rubella cytomegalavirus herpes simplex HIV
434
at what gestation is the risk of congenital rubella highest?
< 3 months
435
what are 3 features of congenital rubella?
Classic triad of - deafness, blindness (congenital cataracts) and congenital heart disease also learning disability
436
what are 6 features of congenital cytomegalovirus?
foetal growth restriction microcephaly hearing loss vision loss learning disability seizures
437
what is the classic triad of congenital toxoplasmosis gondii?
intracranial calcification hydrocephalus chorioretinitis (type of posterior uveitis)
438
what are 6 manifestations of neonatal herpes?
external herpes lesions liver involvement encephalitis sezures, tremmors, lethargy, irritability
439
what are 5 complications of chicken pox in pregnancy?
foetal varicellar zoster syndrome pneumonitis hepatitis encephalitis severe neonatal infection
440
what are 6 mamifestations of congenital varicella syndrome?
foetal growth restriction microcephaly hydrocephalus, learnign disability scars and skin changes limb hypoplasia cataracts and chorioretinitis
441
what is given to a baby immediatly after birth?
vitamin K IM injection
442
what 9 conditions are tested for in the blood spot test?
sickle cell CF congenital hypothyroidism phenylketonuria medium chain acy-COA dehydrogenase deficiency maple syrup urine disease isovaleric acidaemia glutaric aciduria type 1 homocystin
443
at what age does the heel prick test happen?
5 days old
444
how long does the heel prick take to come back?
6-8 weeks
445
when is the NIPE examination done?
<72 hours of birth
446
when is the NIPE repeated?
6-8 weeks by GP
447
when does the ductus arteriosus close?
1-3 days
448
how small should the difference in pre-ductal and post-ductal O2 saturations be in the NIPE?
<2% pre-ductal - R hand post ductal - feet
449
what is the name of oedema of the scalp outside the periosteum due to trauma in birth which crosses suture lines?
caput succedaneum
450
what is the name of a collection of blood between the skull and periosteum due to trauma in delivery which does not cross suture lines?
cephalohaematoma
451
what is erbs palsy?
damage to C5/6 brachial plexus ileading to lweakness in shoulder abduction and external rotation, arm flexion and finger extension may have a 'waiters tip appearance'
452
what are 6 risk factors for neonatal sepsis?
vaginal GBS colonisation GBS sepsis in previous baby Maternal sepsis, chorioamniotitis or fever prematurity premature ROM prolonged rupture of membranes
453
what are 6 red flags for neonatal sepsis?
confirmed/suspected sepsis in mother signs of sock seizures term baby needing mechanical ventilation resp distress starting 4+ hours after burth presumed sepsis in other multiple
454
what are 10 clinical features of neonatal sepsis?
fever reduced tone and activity poor feeding resp distress or apnoea vomiting tachy or brady hypoxia jaundice <24 hours seizures hypoglycaemia
455
what is the nice treatment for neonatal sepsis?
monitor if 1 risk factor/clinical feature start antibiotics within one hour if 2 risk factors/clinical features or 1 red flag
456
what antibiotic should be given in neonatal sepsis?
benzylpenicillin or gentamycin cefotaxime if lower risk
457
what is the ongoing management of neonatal sepsis?
check CRP at 24 hours and blood cultures at 36 hours consider stopping Abx if blood cultures negative and CRP <10 at day 2
458
what counts as extreme preterm?
<28 weeks
459
what counts as very preterm?
28-32 weeks
460
what counts as moderate/late preterm?
32-37 weeks
461
what are 10 early complications of prematurity?
respiratory distress syndrome hypothermia hypoglycaemia poor feeding apnoea and bradycardia neonatal jaundice intraventricular haemorrhage retinopathy of prematurity necrotising enterocolitis immature immune system and infection
462
what are 5 long term complications of prematurity?
chronic lung disease of prematurity learnign an dbehvioural difficulties susceptibility to infections - particularly resp hearing and visual impairement cerebral palsy
463
what is apnoea?
stopping breathing >20 seconds or shorter periods with oxygen desaturation or bradycardia
464
what is the causes of apnoea in neonates?
due to immaturity of the autonomic nervous system
465
what is the management of neonatal apnoea?
tactile stimulation IV caffeine
466
what is the pathophysiology of retinopathy of prematurity?
retinal blood vessel formation is stimulated by hypoxia which is the normal condition of the retina during pregnancy so with early exposure to oxygen in prematurity this can stop retinal blood vessel development and cause retinal detachment
467
who should screening for retinopathy of prematurity be offered to?
<32 weeks gestation <1.5kg
468
what is the management of retinopathy of prematurity?
transpupillary laser photocoagulation cyotherapy injections of intravitreal VEGF inhibitors Surgery if retinal detachment
469
how is a diagnosis of meconium aspiration confirmed?
CXR
470
what are 3 risk factors for meconium aspiration?
gestational age >42 weeks foetal distress APGAR score <7
471
what is the management for suspected HSV encephalitis?
aciclovir
472
How do you get infected with listeria?
consumption of dairy products, raw vegetables, meats and refrigerated foods passed from mother to baby placentally
473
what are clinical manifestations of neonatal listeriosis?
abortion prematurity still birth neonatal sepsis
474
how can listeria infection be diagnosed prenatally and postnatally?
culture or PCR of blood, cervix or amniotic fluid of mother prenatally culture/PCR of blood, CSF, gastric aspirate, meconium of neonate
475
what is the management of neonatal listeriosis?
Ampicillin +/- gentamicin
476
what is maternal hyperthyroidism associated with?
foetal tachycardia small for gestational age prematurity still birth congenital malformations
477
what is maternal hypothyroidism associated with?
lower IQ and impaired psychomotor development
478
what are 6 risk factors for neonatal hypoglycaemia?
IUGR preterm born to mothers with diabetes hypothermic polycythaemic ill for any reason
479
what are 6 symptoms of neonatal hypoglycaemia?
jitters irritability apnoea lethargy drowsiness seizures
480
what counts as hypoglycaemia in neonates?
<2.6 mmol/L
481
what is the pathophysiology of cleft lip and palate?
lip - failure of fusion of the frontonasal and maxillary processes Palate - failure of fusion of palatine processes and nasal septum
482
when is a cleft lip and palate usually fixed?
lip - 3 months palate - 6 months - 1 year
483
what are 2 USS signs in pregnancy of oesophageal atresia?
polyhydramnios no stomach bubble
484
what is a clinical sign of oesophageal atresia in neonates?
persistant salivation and drooling
485
what is the name of a group of midline congenital defects like oesophageal atresia?
VACTERL association Vertebral anorectal cardiac tracheo-oesophageal renal limb anomalies
486
what is the management of oesophageal atresia?
continuous suction of secretions to avoid aspiration until surgery is available
487
what is an exomphalos?
where abdominal contents protrude through umbilical ring covered by transparent sac
488
what congenital condition is duodenal atresia associated with?
downs syndrome
489
what is an x-ray/USS sign of duodenal atresia?
'double bubble' of of distention of stomach and duodenal cap with absence of air distally
490
what are 3 obstetric complications of diabetes in pregnancy?
polyhydramnios preeclampsia early and late foetal loss
491
what are 3 foetal complications of diabetes in pregnancy?
congenital malformations IUGR macrosomia
492
what are 4 neonatal complications of diabetes in pregnancy?
neonatal hypoglycaemia respiratory distress syndrome hypertrophic cardiomyopathy polycythaemia
493
what is scoliosis?
lateral curvature of frontal plane of spine. In severe cases can lead to cardiorespiratory failure from curvature of chest
494
what is torticollis?
Head twisting to the side also called wry neck. The most common cause in infants is a sternomastoid tumour.
495
what is the incubation period for mumps?
14-25 days
496
what is the presentation of mumps?
prodromal fever, muscle aches, lethargy, reduced appetite, headache and dry mouth for a few days followed by parotid swelling usually self limiting after around a week
497
what are 4 complications of mumps?
pancreatitis orchitis meningitis sensorineural hearing loss
498
How is mumps diagnosed?
salivary PCR salivary or blood antibodies
499
what is the management for mumps?
supportive - self limiting infection
500
what is the rash and its spread in chicken pox?
widespread erythematous raised vesicular blistering lesions - starts as macules then papules then vesicles Starts on trunk or face and spreads outwards over 2-5 days the crusts after 5 days
501
what are 5 complications of chicken pox?
bacterial superinfection (necrotising faciitis) dehydration conjunctival lesions pneumonia encephalitis (presents with ataxia)
502
How long can it take someone to become symptomatic after exposure to chicken pox?
10 days - 3 weeks
503
what is the presentation of measles?
fever, coryza and conjunctivitis followed 2 days later by greyish white spots on the buccal mucosa - KOPLIK SPOTS followed by rash 3-5 days post fever, classically starting behind ears self limiting after 7-10 days
504
what sign on examination is pathognomonic for measles?
Koplik spots - grey/white spots on buccal mucosa that appear 2 days after fever
505
what is the rash like in measles and how does it spread?
starts on face (classically behind ears) 3-5 days after fever and then spreads to rest of body erythematous macular rash with flat lesions
506
what is the incubation period of measles?
10-12 days
507
what are 8 complications of measles?
pneumonia diarrhoea dehydration encephalitis meningitis hearing loss vision loss death
508
what pathogen causes scarlet fever?
exotoxins from group a strep (strep pyogenes)
509
what is the rash in scarlet fever and how does it spread?
red-pink blotchy macular rash with rough SANDPAPER skin Starts on trunk and spreads outwards may also have flushed cheeks
510
what are 7 features of scarlet fever?
sandpaper rash fever lethargy flushed face sore throat Strawberry tongue cervical lymphadenopathy
511
what is the management of scarlet fever?
phenoxymethylpenicillin (penicillin V) for 10 days
512
what are 3 conditions associated with scarlet fever?
post-streptococcal glomerulonephritis acute rheumatic fever tonsillitis - strep throat
513
how long is the incubation time for rubella?
2 weeks
514
what is the rash in rubella and how does it spread?
Erythematous macular rash (milder than measles) starts on face and spreads to body lasts 3 days
515
what are 2 rare complications of rubella?
thrombocytopenia encephalitis
516
what are 5 presenting features of rubella?
erythematous macular rash mild fever joint pain sore throat lymphadenopathy
517
what pathogen causes slapped cheek syndrome?
Parvovirus b19
518
what is the presentation of parvovirus B19?
fever, coryza and non-specific viral symptoms followed 2-5 days later by diffuse bright red rash on both cheeks (as though slapped) a few days later a reticular mildly erythematous affecting trunk and limbs - may be raised and itchy
519
what are 4 complications of parvovirus?
aplastic anaemia encephalitis or meningitis pregnancy complications - foetal death rare - hepatitis, myocarditis, nephritis
520
what pathogen causes roseola infantum?
HHV-6/7
521
what is the presentation of roseola infantum?
presents 1-2 weeks after high fever for 3-5 days which the settles and is followed by a rash
522
what is the rash like in roseola infantum?
mild erythematous macular rash on arms, legs, trunk and face that isn't itchy
523
what pathogen causes whooping cough?
bordetella pertussis - gram neg
524
what is the presentation of whooping cough?
mild coryzal symptoms and low grade fever, dry cough followed by more severe coughing fits after 1 week so much so people may vomit, fait or have a pneumothorax
525
what are 3 ways to diagnose whooping cough?
nasopharangeal PCR or culture anti-pertussis toxin IgG if cough present >2 weeks
526
what is the management of whooping cough?
1 - macrolides - azithromycin, erythromycin, clarithromycin within first 21 days 2 - Co-trimoxazole
527
what is a complication of whooping cough?
bronchiectasis
528
what can be given to vulnerable contacts of people with whooping cough?
prophylactic antibiotics
529
what 2 pathogen cause impetigo?
staph aureus - most common strep pyogenes
530
what is the characteristic sign of impetigo?
golden crusted rash/skin infection
531
what are the two different types of impetigo?
bullous - 1-2cm fluid filled vesicles which grow and burst to form golden crusts. may be itchy non-bullous - golden crusts
532
what pathogen always causes bullous impetigo?
staph aureus
533
what is the management of non-bullous impetigo?
1st line - hydrogen peroxide cream 1% 2nd - If unsuitable - topical fusidic acid 3rd - oral flucloxacillin - when more widespread/unwell
534
what is the name of severe bullous impetigo?
staphylococcus scalded skin syndrome
535
what is the management of bullous impetigo?
abx - usually flucloxacillin
536
what are 6 complications of impetigo?
cellulitis sepsis scarring post-streptococcal glomerulonephritis staphylococcus scalded skin syndrome scarlet fever
537
what bacteria causes diphtheria?
corynebacterium diphtheriae
538
what are 5 features of diphtheria?
sore throat + difficulty swallowing fever lymphadenopathy SOB pseudomembrane on tonsils and mucosa of pharynx larynx and nose
539
what is the management of diphtheria?
1 - diphtheria antitoxin Abx - azithromycin, erythromycin, clarithromycin,
540
what is the pathogenic cause of scalded skin syndrome?
staph aureus epidermolytic toxins
541
what age group is usually affected by scalded skin syndrome?
<5 years
542
what sign is positive in scalded skin syndrome?
Nikolysky sign - gentle rubbing of skin causes it to peel away
543
what is a major acute complication of polio?
acute flaccid paralysis
544
what is a possible long term complication of polio?
post-poliomyelitis syndrome - weakness and fatigue of muscle groups affected in acute illness
545
what is the main cause of viral encephalitis in neonates?
HSV-2 - genital herpes
546
what is the most common cause of viral encephalitis?
HSV-1 - from cold sores
547
what is the treatment for encephalitis caused by CMV?
ganciclovir
548
what are 4 complications of encephalitis?
change in personality, memory or cognition leaning disability headache movement disorders
549
what is the most common cause of toxic shock syndrome?
group A strep toxins or s.aureus toxins
550
what are 6 presenting features of toxic shock?
severe diffuse of localised pain in an extremity fever localised swelling or erythema hypotension diffuse, scarlatina-like red rash muscle weakness
551
what is the management of strep toxic shock?
clindamycin + penzylpenicillin or vancomycin
552
what is the management for staph toxic shock?
clindamycin + oxacillin or vancomycin
553
what are 4 complications of toxic shock?
bacteraemia acute resp distress DIC renal failure
554
what is the management for oral candidaiasis?
miconazole gel nystatin suspension fluconazole tablets
555
what does cutaneous candida rash look like?
sore and itchy pustular or papular erythematous rash with an irregular edge and SATELLITE lesions often in flexural areas
556
what is the management of nappy rash?
1 - topical hydrocortisone 1% if inflamed topical imidazoles (clotrimazole), oral antibiotics if severely inflammed
557
what causes hand foot and mouth disease?
coxsackie a virus
558
what is the incubation period for hand foot and mouth?
3-5 days
559
what is the typical history for hand foot and mouth disease?
resp illness + fever 1-2 days later - mouth ulcers then red painful possibly itchy blistering spots across body
560
what is the management for hand foot and mouth?
supportive - will resolve in 7-10 days without treatment
561
what are 3 complications of hand foot and mouth disease?
dehydration bacterial superinfection encephalitis
562
what kind of bacteria in Neisseria meningitidis?
gram negative diplococci
563
what is the most common cause of meningitis in neonates?
group B strep
564
what are 5 non-specific signs of meningitis in neonates?
hypotonia poor feeding lethargy hypothermia bulging fontanelle
565
what is kernig's test?
for meningitis lie patient on back and flex one hip an dknee 90 degrees then straighten knee with hip still flexed - +ve if spinal pain and resistance to movement
566
what is brudzinskis test?
for meningitis lie patient on back and use hand to lift head and neck to flex chin to chest +ve if causes patient to flex hips and knees
567
what is the treatment of sus bacterial meningitis in the community?
benzylpenicillin
568
what is the management of sus bacterial meningitis in <3 months?
cefotaxime + amoxicilin (for listeria)
569
what is the management for sus bacterial meningitis in >3 months?
ceftriaxone + dexomethasone QDS for 4 days
570
what is the prophylaxis for contact with bacterial meningitis?
single dose ciprofloxacin
571
what are 3 common causes of viral meningitis?
HSV enterovirus VZV
572
where should an LP be performed?
L3/4
573
what is the CSF picture in bacterial meningitis?
cloudy high protein Low glucose high neutrophils culture +ve
574
what is the CSF picture in viral menigitis?
clear mild raise/normal protein normal glucose high lymphocytes -ve culture
575
what are 5 complications of meningitis?
hearing loss seizures cognitive impairment memory loss cerebral palsy
576
what are 7 causes of cerebral palsy?
antenatal - maternal infection, trauma in preggo perinatal - birth asphyxia, pre-term birth postnatal - meningitis, severe neonatal jaundice, head injury
577
what are the 4 types of cerebral palsy?
spastic dyskinetic ataxic mixed
578
what is spastic cerebral palsy?
hypertonia and reduced function due to UMN damage
579
what is dyskinetic cerebral palsy?
problems controlling muscle tone with hyper and hypotonia casing athetoid (slow writing) movements and oro-motor problems due to basal ganglia damage
580
what is ataxic cerebral palsy?
problems with coordinated movement due to cerebellar damage
581
what are 6 signs of cerebral palsy?
failure to meet milestones increased/decreased tone hand preference <18 months problems with coordination, speech or walking feeding or swallowing problems learning difficulties
582
what are 6 complications of cerebral palsy?
learning difficulty epilepsy kyphoscoliosis muscle contractures hearing/visual impairement GORD
583
what is the management for cerebral palsy?
multidisciplinary physio OT speech and language dietician orthopaedics Meds - muscle relaxants - baclofen, antiepileptics, glycopyrronium bromide - drooling
584
what age group do febrile convulsions occur in?
ages 6 months - 5 years
585
what are simple febrile convulsions?
one generalised tonic clonic <15 minutes
586
what are 5 possible causes of global developmental delay?
downs fragile X foetal alcohol syndrome rett syndrome Metabolic disorders
587
what are 5 possible causes of gross motor delay?
cerebral palsy ataxia myopathy spina bifida visual impairement
588
what are 5 possible causes of fine motor delay?
dyspraxia cerebral palsy muscular dystrophy visual impairment congenital ataxia
589
what are 6 possible causes of language delay?
social circumstance hearing impairement learning disability neglect autism cerebral palsy
590
what are 3 possible causes of social delay?
emotional and social neglect parenting issues autism
591
what are the 4 areas of development?
gross motor fine motor and vision language and hearing personal and social
592
what is the gross motor development of a 4 month old?
start supporting head and keep in line with body
593
what is the gross motor development at 6 months?
Maintain sitting position usually supported as unbalanced
594
what is the gross motor development at 15 months?
walk unaided
594
what is the gross motor development at 9 months?
sit unsupported start crawling maintain standing and bouncing position wile supported
594
what is the gross motor development at 12 months?
standing and cruising
594
what is the gross motor developement at 18 months?
squat and pick things up
595
what is the gross motor development at 2 years?
run and kick ball
596
what is the gross motor development at 3 years?
climb stairs one foot at time stand on one leg ride tricycle
597
what is the gross motor development of a 4 year old?
hop climb stairs normally
598
what are 8 red flags of developemental milestones?
lost development unable to hold object 5 months not sitting unsupported at 12 months not standing independently 18 months not walking 2 years not running 2.5 years no words 18 months no interest in others 18 months
599
what is social development at 6 weeks?
smiles
600
what is social development at 3 months?
communicates pleasure
601
what is social development at 6 months?
curious and engages with people
602
what is social development at 9 months?
apprehensive around strangers
603
what is social development at 12 months
engages with others pointing and handing objects waves bye claps
604
what is social development at 2 years?
interest in others beyond parents parallel play
605
what is social development by 3 years?
play with others bowel control
606
what is social development by 4 years?
has best friend dry at night dresses self imaginative play
607
what is language development at 3 months?
cooing
608
what is language development at 9 months?
babbling
609
what is language development at 12 months?
say 1 word in context follows simple instructions
610
what is language development at 18 months?
5-10 words
611
what is language development at 2 years?
combines 2 words
612
what is language development at 3 years?
basic 3 word sentences
613
what is language development at 4 years?
tells stories
614
what is fine motor development at 8 weeks?
fixes and attempts to follow recognises faces
615
what is fine motor development at 6 months?
palmar grasp
616
what is fine motor development at 9 months?
scissor grasp between thumb and forefinger
617
what is fine motor development at 12 months?
pincer grasp with tip of thumb and forefinger scribbles randomly
618
what is fine motor development at 14-18 months?
clumsily use cutlery tower of 2-4 blocks
619
what is the first and second line management for tonic-clonic seizures?
1 - sodim valporate 2 - lamotrigine or carbamezapine
620
what is the 1st and 2nd line management for focal seizures?
1 - carbamexapine or lamotragine 2 - sodium valporate or levetiracetam (keppra)
621
where do focal seizures start?
temporal lobes
622
what is the first line management for absence seizures?
ethosuximide
623
what are atonic seizures?
drop attacks with breif lapses of muscle tone usually lasting <3 mins may be indicative of lennox-gastaut syndrome
624
what is the 1st and 2nd line management of atonic seizures?
1 - sodium valporate 2 - lamotrigine
625
what is the management of myoclonic seizues?
1 - sodium valporate others - lamotrigine, levetiracetam or topiramate
626
when do infantile spasms usually start?
around 6 months
627
what is the prognosis for infantile spasms?
1/3rd die by 25 1/3rd keep seizures 1/3rd seizure free
628
what are 2 treatment options for infantile spasms?
pred vigabitrin
629
what are 4 side effects of sodium valporate?
teratogenic liver damage and hepatitis hair loss tremor
630
what are 3 side effects of carbamazepine?
agranulocytosis aplastic anaemia P450 drug interactions
631
what are 3 side effects of phenytoin?
folate and vitamin d deficiency megaloblastic anaemia osteomalacia
632
what are 2 side effects of ethosuximide?
night terrors rashes
633
what are 2 side effects of lamotrigine?
stevens-johnson syndrome leukopenia
634
what is status epilepticus?
seizure lasting >5 mins 2 or more seizures without regaining consciousness
635
how is status epilepticus managed?
IV lorazepam buccal midazolam rectal diazepam IV phenytoin can be used if seizure persists
636
what chromosome is affected in sickle cell anaemia?
chromosome 11
637
what are 10 complications of sickle cell?
anaemia increased risk of infection chronic kidney disease sickle cell crisis acute chest syndrome stroke avascular necrosis pulmonary hypertension gallstones priaprism (painful persistent erections)
638
what are 4 things that can trigger sickle cell crisis?
dehydration infection stress cold weather
639
what is the management of sickle cell crisis?
low threshold for admission treat infections keep warm good hydration analgesia
640
what is vaso-occlusive crisis?
sickle shaped RBCs clog capillaries causing distal ischaemia presents with pain and swelling in hands, feet, chest or back can cause priapism in men
641
what is splenic sequestration crisis?
blood flow to spleen blocked by sickle cells causing acutely enlarged painful spleen which can lead to severe anaemia and hypovolaemic shock management - supportive, blood transfusion, fluid resus
642
what is an aplastic crisis?
complication of sickle cell temporary absence of creation of RBCs usually triggered by parvovirus B19
643
what is acute chest syndrome?
vaso-oclussive crisis due to sickle cells of the blood vessels of the lungs causing fever, SOB, chest pain, cough and hypoxia
644
what is the management of acute chest syndrome?
analgesia good hydration Abx blood transfusion for anaemia incentive spirometry resp support
645
what is the management for sickle cell?
avoid triggers vaccines Abx prophylaxis (penicillin V) hydroxycarbamine crizanluzumab blood transfusion bone marrow transplant
646
what medication can be used in sickle cell to stimulate foetal haemoglobin production?
hydroxycarbamide
647
what are 8 signs of thallasaemia?
microcytic anaemia splenomegally pronounced forehead and malar eminences jaundice gallstones poor growth and development fatigue pallor
648
what investigation can be used to detect thallasaemia?
haemoglobin electrophoresis
649
what are the two types of thalassaemia?
alpha or beta
650
what is thalassaemia minor?
carriers with one faulty gene causes mild microcytic anaemia usually only requires monitoring
651
what is thalassamia intermedia?
either two defective geners or one and one deletion causes more significant microcytic anaemia patients require monitoring and blood transfusions which may require iron chelation
652
what is thalassaemia major?
homozygous for the deletion genes severe anaemia and failure to thrive in early childhood severe microcytic anaemia splenomegaly bone deformities regular transfusions, iron chelation, splenectomy
653
what is klinefelter syndrome?
when a male has a additional X chromosomes making them 47XXY
654
what is the presentation of Kleinfelters syndrome? (8)
normal development as male till puberty then taller height wider hips and gynaecomasitia weaker muscles small testes reduced libido shyness infertility subtle learning difficulties
655
what is the management of kleinfelters syndrome?
testosterone injections advanced IVF techniques breast reduction
656
what are 4 things that at at increased risks in kleinfelters?
breast cancer (more than other men) osteoporosis diabetes anxiety and depression
657
what is turners syndrome?
females with single X chromosome on chromosome 45 -> 45XO
658
what are 9 features of turner syndrome?
short stature webbed nack high arching palate downward sloping eyes with ptosis broadchest and with wide spaced nipples cubitus valgus underdeveloped ovaries late/incomplete puberty infertile
659
what are 9 conditions associated with turners syndrome?
recurrent otitis media recurrent UTI Cardiac - coarctation of aorta, pulmonary stenosis hypothyroid hypertension obesity diabetes osteoporosis learning difficulties
660
what are 3 managements for turners?
growth hormone therapy for height oestrogen and progesterone for female secondary sex characteristics fertility treatment
661
what are 8 dysmorphic features of downs syndrome?
hypotonia brachycephaly (small head with flat back) short neck short stature flattened face and nose prominent epicanthic folds upward sloping palpebral fissures single palmar crease
662
what are 8 complications of downs?
recurrent otitis media and deafness (eustachion tube abnormalities) visual problems hypothyroidism cardiac defects - ASD, VSD, PDA, tetralogy atlantoaxial instability leukaemia - AML (most common) and ALL Alzheimers Hirschprungs disease and duodenal atresia
663
what cardiac defects are more likely in downs?
ASD VSD PDA Tetralogy of fallot
664
what nuchal thickness is indicative of downs?
>6mm
665
what is the combinded test for downs syndrome?
11-14 weeks USS for neucal transleucency bloods - B-HCG, PAPPA
666
what is the tripple test?
between 14-20 weeks maternal bloods - B-HCG, alpha-fetoprotein, serum oestrodiol
667
what is the quadruple test for downs syndrome?
14-20 weeks HCG, AFP, Total Oestrodiol + inhibin-A
668
what are 2 ways of antenatal testing for downs?
chorionic villus sampling amniocentesis
669
what are 4 routine follow ups needsd in downs?
regular thyroid checks - 2 yearly echo regular audiometry regular eye checks
670
what is the average life expectancy for downs?
60 years
671
what is the genetics underlying patau syndrome?
trisomy 13
672
what genetics cause Edwards syndrome?
trisomy 18
673
What are 7 key features of Edwards syndrome syndrome?
Rocker(rounded)-bottom feet low birth weight prominent occiput small mouth and chin short sternum flexed overlapping fingers cardiac and renal malformations
674
what are 6 features of Pataus?
structural defects of brain scalp defects small eyes and eye defects cleft lip and palate polydactyly cardiac and renal malformations
675
what gene is affected in fragile X syndrome?
fragile X mental retardation 1 gene on X chromosome males are always affected but females vary
676
what are 8 features of fragile X?
intellectual disability long narrow face large ears large testes after puberty hypermobile joints ADHD ASD Seizures
677
what gene is affected in Angelman syndrome?
UBE3A gene on chromosome 15
678
what are 6 key features of Angelman syndrome?
fascination with water happy demeanour wide spaced mouth and teeth learning and developmental delay Fair skin, light hair, blue eyes epilepsy
679
what is the genetics of prader-willi?
loss of functional genes on proximal arm of chromosome 15 inhertied from father
680
what are 6 key features of prader-willi?
constant insatiable hunger hypotonia mild/moderate learnign disability hypogonadism dysmorphic - narrow forehead, almond eyes, thin upper lip, downturned mouth mental health problems
681
what is the management of prader willi?
growth hormone - for improving muscle development an body composition dieticians, education support, psych, physio, OT
682
what are 8 features of noonan syndrome?
short stature broad forehead downward sloping eyes with ptosis hypertelorism (widespaced eyes) prominent nasolabial folds low set ears webbed neck widely spaced nipples
683
what are 6 conditions associated with noonan syndrome?
congenital heart disease Undescended testes learning disability lymphoedema increased risk of leukaemia and neuroblastoma
684
what is the cause of william syndrome?
deletion on chromosome 7 - usually random rather than inherited
685
what are 8 features of william syndrome?
broad forehead starburst iris flattened nasal bridge long philtrum wide mouth and spaced teeth small chin sociable and trusting personality mild learning disability
686
what are 4 conditions associated with william syndrome?
supravalvular aortic tenosis hypercalcaemia ADHD hypertension
687
what is the management for williams sydrome?
echo and BP monitoring low calcium diet
688
what is gower's sign?
children with proximal weakness get onto their hands and knees then go into downward dog and push themselves up using their hands MUSCULAR DYSTROPHY
689
what is the name of the sign that is the way children with muscular dystrophy get up?
Gower's sig
690
what are 7 types of muscular dystrophy?
duchennes muscular dystrophy beckers muscular dystrophy faciosapulohumeral muscular dystrophy oculopharyngeal muscular dystrophy limb-girdle muscular dystrophy emery-dreifuss muscular dystrophy
691
what is the inheritance for duchennes MD?
X-linked recessive
692
what is the prognosis for duchennes MD?
25-35 years
693
what is the management of duchennes MD?
oral steroids - slow progress of muscle weakness creatine supplements improve muscle strength
694
when do symptoms start to appear in duchennes MD?
3-5 years
695
when do symptoms start to appear in beckers MD?
8-12 years
696
when does myotonic dystrophy usually present?
adulthood
697
what are 4 key features of myotonic dystrophy?
progressive muscle weakness prolonged muscle contractions cataracts cardiac arrhythmia
698
what are 4 key features of facioscapulohumeral muscular dystrophy?
weakness around face progressing to shoulders and arms sleeping with eyes slightly open weak pursing lips unable to blow out cheeks without air leaking from mouth
699
what are 3 key features of oculopharyngeal MD?
bilateral ptosis restricted eye movements swallowing problems usually presents in alate adulthood
700
what is limb girdle MD?
presents in teenage years with progressive weakness around limb girdles - hips and shoulders
701
what is emery-dreifuss MD?
presents in childhood with contractures which restricts range of movement also progressive weakness and wasting of muscles starting with upper arms and lower legs
702
what chromosome and what gene is affected in CF?
chromosome 7 cystic fibrosis transmembrane conductance regulatory gene
703
what does the CF transmembrane conductance regulatory gene code for?
Chloride channels
704
what are 3 features of CF?
thick pancreatic and biliary secretions causing blockages low vllume thick airway secretions congenital bilateral absence of vas deferens
705
what is the inheritance of CF?
autosomal recessive
706
what are 7 causes of clubbing in children?
hereditary clubbing cyanotic heart disease infective endocarditis CF TB IBD Liver cirrhosis
707
what are 3 key tests for CF?
newborn blood spot test sweat test - GOLD genetic testing
708
what is the diagnostic chloride conc for CF on sweat test?
60 mmol/L
709
what is a key problematic coloniser for people with CF?
pseudomonas aeruginosa
710
what is treatment for pseudomonas infection in CF?
long termtobramycin nebs or oral ciprofloxacin
711
what is the management for CF?
multidisciplinary chest physio exercise high calory diet creon tablets prophylactic flucloxacillin tx infections bronchodilators DNase Nebs hypertonic saline nebs vaccination
712
what is the median life expectancy for CF?
47 years
713
what bacteria causes TB?
mycobacterium tuberculosis
714
what stain is required in TB?
Zeihl-neelson stain acid-fast bacilli that turn red against blue background on Z-N
715
what are 8 symptoms of TB?
cough haemoptysis lethargy fever/night sweats wt loss lymphadenopathy erythema nodosum spinal pain
716
what are 2 investigations for TB?
mantoux test interferon gamma release assay
717
what is the mantoux test?
for TB inject tuberculin proteins into intradermal space and measure size of injection site at 72 hours - >5mm = positive
718
what is the appearance of disseminated miliary TB on CXR?
millet seeds uniformally distributed
719
what is the treatment for active TB?
RIPE Rifampicin - 6 months Isoniazid - 6 months Pyrazinamide - 2 months Ethanbutol - 2 months
720
what is the treatment of latent TB?
isoniazid and rifampicin 3 months or isoniazid for 6 months
721
what are 3 side effect of rifampicin?
red/orange piss and tears reduced CYP450 drug effectiveness - COCP hepatotoxic
722
what should be prescribed with isoniazid?
pyridoxine (vit B6)
723
what are 2 side effects of isoniazid?
peripheral neuropathy hepatotoxic
724
what are 2 side effects of pyrazinamide?
hyperuricaemia => gout and kidney stones hepatotoxic
725
what are 2 side effects of ethambutol?
colour blindness reduced visual acuity
726
what are 3 risks of undescended testes?
testicular torsion infertility testicular cancer
727
what are 5 risk factors for undescended testes?
FHx low birth weight small for gestational age prematurity maternal smoking
728
what is the management for unilateral undescended testes?
watch and wait for 3 months then refer orchidopexy between 6-12 months
729
what is the usual presentation of testicular torsion?
teenage boy unilateral testicular pain abdo pain and vomiting often triggered by sport
730
what are 5 examination findings in testicular torsion?
firm swollen testicle elevated esticle absent cremasteric reflex abnormal testicular lie rotation so epididymis is not posterior
731
what is the name of the deformity that makes testicular torsion more likely?
bell-clapper deformity - testicle hangs in more horizontal position
732
what is the management for testicular torsion?
nil by mouth analgesia urgent senior urology assessment surgical exploration of scrotum orchiopexy orchidectomy if needed
733
what sign may be seen on USS for testicular/ovarian torsion?
whirlpool sign - spiral appearance of spermatic cord and blood vessels
734
what are 6 presentations of congenital hypothyroidism?
usually picked up on blood spot prolonged neonatal jaundice poor feeding constipation increased sleeping reduced activity slow growth and development
735
what antibody is present in hashimotos?
antithyroid peroxidase antibodies and antithroglobulin antibodies
736
what causes congenital adrenal hyperplasia?
congenital deficiency in 21-hydroxylase enzyme causing underproduction of cortisol and aldosterone and overproduction of androgens (testosterone) Causes increased sodium and potassium excretion
737
what is the inheritance pattern for congenital adrenal hyperplasia?
autosomal recessive
738
what is the presentation of congenital adrenal hyperplasia in severe cases?
enlarged clitoris in females hyponatraemia hyperkalaemia hypoglycaemia poor feeding vomiting dehydration arrhythmias SKIN HYPERPIGMENTATION
739
what is the presentation of congenital adrenal hyperplasia in mild cases?
female - tall for age - facial hair - absent periods - deep voice - early puberty Males - tall or age - deep voice - large penis - small testicles - early puberty
740
what is the management of congenital adrenal hyperplasia?
cortisol replacement - hydrocortisone aldosterone replacement - fludrocortisone virilised genitals corrective surgery
741
what is the inheritance pattern for androgen insensitivity syndrome?
X-linked recessive due to mutation on androgen receptor gene on X-chromosome in XY males
742
what is the pathophysiology of androgen insensitivity syndrome?
cells are unresponsive to androgen hormones due to a lack of androgen receptors leading to excess androgens which are converted to oestrogen resulting in female secondary sexual characteristics and external female phenotypes from birth. Internally there are testes in the abdo/inguinal canal but no internal female organs due to the testes releasing anti-Mullerian hormone
743
what are 2 presentations of androgen insensitivity syndrome?
inguinal hernias primary amenorrhoea
744
what will hormone bloods be like in androgen insensitivity syndrome?
Raised LH normal/raised FSH Normal/raised testosterone for a man Raised oestrogen for a man
745
what is the management for androgen insensitivity syndrome?
bilateral orchidectomy - testicular tumours oestrogen therapy vaginal dilators and surgery
746
what age does Wilms tumours usually present in?
<5 years
747
what are 7 presentations of Wilms tumours?
abdo pain haematuria lethargy fever HTN Weight loss Abdo mass
748
What is the first line Ix for a Wilms tumour?
USS abdo
749
what is the management of Wilms tumour?
surgical excision +/- nephrectomy +/- adjuvant chemo/radio
750
what is the prognosis for Wilms tumour?
up to 90% cure in early stages
751
what is the most common leukaemia in children?
1st - ALL 2nd - AML
752
what age is the peak incidence of ALL?
2-3 years
753
what age is the peak incidence of AML?
<2 years
754
what are 4 conditions that are risk factors for leukaemia?
Down syndrome kleinfelter syndrome Noonan syndrome faconi's anaemia
755
what is the prognosis for ALL?
80% cure rate
756
what are 7 complications of chemo?
failure of treatment stunted growth and development immunodeficiency and infection neurotoxicity infertility secondary malignancy cardiotoxicity
757
what causes Idiopathic thrombocytopenic purpura?
type 2 hypersensitivity reaction that casues antibodies to target and destroy platelets either spontaneously or triggered due to viral infection
758
what age group does idiopathic thrombocytopenic purpura present in?
<10 years
759
what is the management for severe idiopathic throbocytopenic purpura?
prednisolone IVIG blood transfusion if required platelet transfusion may work temporarily
760
what are 4 complications of ITP?
chronic ITP anaemia intracrania and subrachnoid haemorrhage GI bleeds
761
what are 5 causes of anaemia in infants?
physiological anaemia anaemia of prematurity blood loss haemolysis twin-twin transfusion syndrome
762
what is physiological anaemia of infancy?
normal dip in haemoglobin at 6-9 weeks due to high O2 at birth
763
what are 4 reasons for anaemia of prematurity?
less time in utero getting iron from mum RBC production cant keep up with growth reduced EPO blood tests
764
what is the treatment for helminth infection?
albendazole or mebendazole
765
what can interfere with iron absorption?
acid from stomach is required to comvert iron into ferrous (Fe2+) form so PPIs can interfere with absorption also coeliac or chrons
766
what test can be used to identify haemolytic disease of the newborn?
direct coombs test
767
what deficiency causes haemophilia A?
factor VIII
768
what deficiency causes haemophilia B?
factor IX
769
what is the inheritance pattern for haemophilia?
X-linked recessive
770
what are 3 complications of haemophilia?
intracranial haemorrhage haemarthrosis compartment syndrome
771
what is von willebrand factor?
a glycoprotein important in platelet adhesion and aggregation (formation of platelet plug)
772
what are the 3 types of von-willebrand factor disease?
1 - partial deficiency 2 - reduced function 3 - complete deficiency
773
what is the acute management of von willebrand disease?
desmopressin (stimulated release of vWF from endothelial cells) Tranexamic acid vWF infusion +/- factor VIII
774
what are 5 options for heavy menstrual periods?
tranexamic acid mefanamic acid mirena coil COCP norethisterone
775
what is faconi anaemia?
rare inherited disorder of gradual bone marrow failure and birth defects eventually leading to aplastic anaemia
776
what is kallmann syndrome and 3 features?
an X linked disorder Causes hypogonadotrophic hypogonadism due to failure of GnRH release Delayed puberty Low sex hormones, LH and FSH differentiating feature of INABILITY TO SMELL
777
what are the three key areas affected by Autism?
deficits in social interaction communication and behaviour
778
what are 6 possible social diferences in autism?
lack of eye contact delay in smiling avoids physical contact unable to read non-verbal cues difficulty establishing friendships no desire to share attention (play with others)
779
what are 4 communication diferences in autism?
delay, absence or regression in language lack of appropriate non-erbal communication difficulty with imaginative or imitative behaviours repetitive use of words or phrases
780
what are 6 behavioural differences in autism?
greater interest in things than people sterotypical repetitive movements intensive deep interests repetitive behaviours and fixed routines anxiety and distress with deviation from routine extremely restricted food preferences
781
what are 6 features of ADHD?
very short attention span quick moving from one activity to another quick loss of interest in task or inability to persist with challenging task constantly moving or fidgeting impulsive behaviour disruptive or rule breaking
782
what are 3 ADHD meds?
methyphenidate - ritalin dexamfetamine atomoxetine
783
what are 8 features of annorexia?
excessive wt loss amenorrhoea lanugo hair hypokalaemia hypotension hypothermia changes in mood cardiac complications
784
what are 3 cardiac complications of anorexia?
arrythmias cardiac atrophy sudden cardiac death
785
what are 7 features of bulimia?
alkalosis on Blood gas hypokalaemia erosion of teeth swollen salivary glands mouth ulcers GORD calluses on knuckles (russels sign)
786
what are 3 micronutrient deficiencies due to starvation in eating disorders?
hypomagnesaemia hypokalaemia hypophosphataemia
787
what is the management of eating disorders to avoid refeeding syndromes?
slowly refeed magnesium, potassium, posphate and glucose monitoring fluid balance monitoring ECG monitoring supplementations with electrolytes, B vitamins and thyamine
788
what is the first line antidepressant in children?
Fluoxetine 10-20mg
789
what are 8 signs of dehydration?
appear unwell altered consciousness sunken eyes tachycardia tachypnoea reduced skin turgor dry mucous membranes decreased urine outpt
790
what is the first line maintenance fluid choice in children >28 days?
0.9% NaCl + 5% glucose
791
what is the first line fluid choice in neonate <28 days?
if well 10% dextrose if unwell seek advice
792
what is the formula for maintenance fluids in >28 days of life?
100 mk/kg/day 1st 10kg 50ml/kg/day 2nd 10kg 20 ml/kg/day >20kg
793
what is the maintenance fluid requirements for <28 days?
day 1 - 50-60 ml/kg/day 2 - 70-80 ml/kg/day 3 - 80-100 ,l/kg/day 4 - 100-120 ml/kg/day 5-28 - 120-150 ml/kg/day
794
what is the calculation for percentage dehydration?
(well weight - current weight)/well weight X100
795
how do you calculate fluid deficit?
% dehydration X weight (kG) X 10
796
how do you calculate total fluid requirement?
maintenance fluid + fluid deficit
797
what fluids should be given for resucitation?
0.9% NaCl 10 ml/Kg <10 mins
798
what is the normal ages to start to develop secondary sexual characteristics in males and females?
males - 9 years females - 8 years development before this age is precocious puberty
799
what are are 8 complications of obesity?
Slipped upper femoral epiphysis (SUFE) idiopathic intracranial hypertension hypoventilation syndrome fatty liver disease T2DM PCOS HTN abnormal blood lipids
800
what medication can be given to severely obese children >12 years?
orlistat
801
what are 4 causes of gonadotrophin (pituitary) dependant precocious puberty?
idiopathic/familial CNS abnormalities - congenital, tumours (neurfibromatosis, craniopharyngioma) Hypothyroism
802
what are 5 gonadotrophin independent causes of precocious puberty?
adrenal tumour congenital adrenal hyperplasia ovarian/testicular tumour (granulosa/leydig cell) Exogenous sex steroids
803
what age is usually affected by premature breast development (thelarche) absent of any other signs of precocious puberty?
6 months - 2 years fluctuating development of breast buds which is self limiting and does not require treatment
804
what is premature pubarche (adrenarche)?
when pubic hair develops precociously with no other signs of sexual development most commonly caused by accentuation of normal maturation androgen. More common in non-white population. Leads to increased risk of PCOS in adult life.
805
what is delayed puberty in males and females?
>14 years in females >15 years in males
806
what are 7 causes of delayed puberty?
congenital/familial - most common hypogonadotrophic hypogonadism Hypergonadotrophic hypogonadism
807
what are 4 causes of hypogonadotrophic hypogonadism?
systemic disease - CF, asthma, crohns Hypothalamo-pituitary disorders - tumours, kallman syndrome, pituitary dysfunction acquired hypothyroidism
808
what are 3 causes of hypergonadotrophic hypogonadism?
chromosomal abnormalities - klinefelter syndrome, turner syndrome steroid hormone enzyme deficiencies acquired gonadal damage
809
what is the most common solid tumour in children?
brain tumour
810
what is the most common brain tumour in children?
astrocytomas - raneg from benign to the highly malignant glioblastoma multiforme
811
what is the clinical presentation of brain tumour?
persistant/recurrent vomiting problems with balance, coordination or walking behaviour change abnormal eye movement seizures abnormal head positioning headache blurred/double vision lethargy deteriorating school work/developmental delay increasing head size in infants
812
what is medulloblastoma?
2nd most common childhood brain tumour arises in midline of posteriod fossa and may spread through CSF to spinal mets
813
what is an ependymoma?
brain tumour that behaves much like medulloblastoma but arises in posterior fossa
814
what is a brainstem glioma?
malignant brain tumour with very poor prognosis
815
what is a craniophryngioma?
developmental tumour arising from squamous remnant of rathke pouch - non-malignant but locally invasive
816
what age group is affected by neuroblastoma?
<6 years
817
where do neuroblastomas arise from?
neural crest tissue in adrenal medulla and sympathetic nervous system
818
what is the presentation of neuroblastoma?
mostly abdo mass but primary tumour may be anywhere along sympathetic chain from neck to pelvis pallor, wt loss, abdo mass, hepatomegally, bone pain, limp
819
what investigations can be does for neuroblastoma?
urinary catecholamine metabolite levels (VMA/HVA) BIOPSY
820
what is the inheritance pattern for retinoblastomas and what chromosome is it found on?
autosomal dominant - incomplete penetrance chromosome 13
821
what are 2 presenting features of retinoblastoma?
red reflex turns white squint
822
what is the most common liver tumour in children?
hepatoblastoma usually presents with bloating and abdo mass
823
when does the palmar reflex usually disappear?
2-6 months
824
when does the sucking reflex usually start?
around 32 weeks gestation - not fully developed until 36 weeks
825
how long does the moro reflex last?
around 2 months
826
how long does the stepping reflex last?
around 2 months
827
how long does the rooting reflex last?
around 4 months
828
when does osteosarcoma usually present?
10-20 year olds
829
what is the most common bone to be affected by osteosarcoma?
femur tibia and humorous also common
830
what are 5 symptoms of bone tumour?
persistent bone pain pain worse at night - disturb or wake from sleep bone swelling palpable mass restricted joint movement
831
what is a classical x-ray finding of bone tumour?
periosteal reaction causing 'sun-burst' appearance of bone
832
what can be seen on LFTs that suggest bone tumour?
raised ALP
833
what are 2 complications of bone tumours?
pathological fractures metastasis
834
what is the 1st line investigation for bone tumour?
urgent (in 48 hours) x-ray and specialist assesment
835
what is the most common bone cancer in children?
osteosarcoma
836
what is the second most common bone cancer in children?
ewing sarcoma
837
what does use of NSAIDs in chicken pox increase risk of?
necrotising fasciitis - increase risk of bacterial infection
838
what is the infectivity period of chicken pox?
from 4 days pre-rash to around 5 days after rash appears when all crusted over
839
when is the meningitis B vaccine given?
2 months, 4 months, 12 months
840
what is the proper name for threadworms?
enterobius vermicularis
841
what is the management for threadworms?
Mebendazole single dose for whole household only for >6 months
842
what does a blood gas with pyloric stenosis look like?
Hypochloraemia Hypokalaemia elevated bicarb
843
what are 5 causes of obesity in children?
downs syndrome prader-willi syndrome growth hormone deficiency hypothyroid cushings syndrome
844
what are 5 consequences of obesity in children?
Ortho - SUFE, MSK Pains, Blounts disease Psycho - bullying, low self esteem sleep apnoea benign intracranial hypertension long term - T2DM, hypertension, IHD
845
what is the first line management for tonic clonic seizures in kids?
sodium valporate
846
what is the second line management for tonic clonic seizures in kids?
lamotrigine or levetiracitam
847
where in the brain do focal seizures start?
temporal lobes affect hearing, speech, memory and emotion may cause people to do things on autopilot
848
what is the management for focal seizures?
1 - lamotrigine or levitiracitam 2 - carbamazepine, oxcarbazepine, zonisamide. 3 - lacosamide
849
what is the 1st line management for absence seizures?
1 - ethosuximide 2 - sodium valporate/lamotrigine/levetiracitam
850
what syndrome is atonic seizures linked to?
Lennox-Gastaut syndrome
851
what is the mangement for atonic seizures?
1 - sodium valporate 2 - lamotrigine
852
what is the 1st line management for myoclonic epilspsy?
sodium valporate/levetiracitam (girls)
853
what is the 2nd line management for myoclonic seizures?
lamotrigine, levetiracetam or topiramat
854
what is the management for infantile spasms?
Prednisolone Vigabatrin
855
what are 4 side effects of sodium valporate?
teratogenic liver damage/hepatitis Hair loss tremmor
856
what is the MOA of sodium valporate?
increases activitiy of GABA
857
what are 3 side effects of carbamezapine?
Agranulocytosis Aplastic anaemia Induces the P450 system so there are many drug interactions
858
what are 3 side effects of phenytoin?
Folate and vitamin D deficiency Megaloblastic anaemia (folate deficiency) Osteomalacia (vitamin D deficiency)
859
what are 2 side effects of ethosuximide?
Night terrors Rashes
860
what are 2 side effects of lamotrigine?
Stevens-Johnson syndrome or DRESS syndrome. These are life threatening skin rashes. Leukopenia
861
what is the management of status epilepticus in hospital?
IV lorazepam x2 IV phenytoin/phenobarbital ITU
862
what is the medical management of seizure in the community?
Buccal midazolam Rectal diazepam
863
what inheritance pattern is there in achondropasia?
autosomal dominant
864
what mutation causes achondroplasia?
mutation in the fibroblast growth factor receptor 3 (FGFR-3) gene
865
what are 5 features of achondroplasia?
short limbs (rhizomelia) with shortened fingers (brachydactyly) large head with frontal bossing and narrow foramen magnum midface hypoplasia with a flattened nasal bridge 'trident' hands - gap between middle and ring finger lumbar lordosis
866
what is the management of asthma <5 years?
SABA + ICS for 8 weeks + leukotriene receptor antagonist (LTRA) refer to secondary care
867
what is a paeds ICS low dose?
<200 micrograms
868
what is a paeds ICS high dose?
>400 micrograms
869
what are 2 conditions associated with hypospadias?
cryptorchidism inguinal hernia
870
what medication can be used in CF patients who are homozygous for the delta F508 mutation?
Lumacaftor/Ivacaftor (Orkambi)
871
what is a venous hum?
innocent murmur heard just below clavicles of blood returning to heart
872
what is stills murmour?
innocent murmur of Low-pitched sound heard at the lower left sternal edge
873
what are 8 features of innocent murmurs?
soft-blowing murmur in the pulmonary area or short buzzing murmur in the aortic area may vary with posture localised with no radiation no diastolic component no thrill no added sounds (e.g. clicks) asymptomatic child no other abnormality
874
what is the management of asymptomatic neonatal hypoglycaemia?
encourage feeding + monitor
875
what is the management of symptomatic neonatal hypoglycaemia?
SCBU IV 10% dextrose
876
Is perthes usually unilateral or bilateral?
unilateral - only bilateral 10% of time
877
How is perthes disease diagnosed?
X-ray
878
what vaccines are given at 8 weeks?
6 in 1 (DTaP, IPV, Hib, Hep B), Men B Rotavirus
879
what vaccines are given at 12 weeks?
6 in 1 (DTaP, IPV, Hib, Hep B) Pneumococcal Rotavirus
880
what vaccines are given at 16 weeks?
6in1(DTaP,IPV,Hib,HepB) MenB.
881
what vaccines are given at 1 year?
Hib/Men C Pneumococcal booster MMR Men B booster.
882
what vaccines are given at 3 years (4 months)?
DTap/IPV - Diptheria, Tetenus, Petussus, Polio MMR.
883
what vaccine is given at 12-13 years?
HPV
884
what vaccines are given at 14 years?
Tetanus, diphtheria, polio Men ACWY.
885
what is in the 6 in 1 vaccine?
Diphtheria Tetanus Pertussis Hib Hepatitis B Inactivated Polio Vaccine
886
when is the 6 in 1 vaccine given?
8, 12 and 16 weeks
887
when is the MMR vaccine given?
12 months and 3 years
888
When is the rotavirus vaccine given?
8 and 12 weeks
889
when is the men B vaccine given?
8 and 16 weeks booster at 1 year
890
what are the two main sanctuary sites for leukaemia?
CNS Testes most likely to have secondary malignancies
891
what is the dose of insulin infusion in DKA?
0.1 units/kg/h
892
what are 6 blood results that might be seen in DKA?
Acidotic Hyperglycaemia Ketonaemia Low Bicarb raised creatinine raised potassium
893
what is a key complication of rapid correction of DKA?
Cerebral oedema
894
what is the management for cerebral oedema?
slowing IV fluids IV mannitol IV hypertonic saline
895
what is mild DKA?
pH 7.2- 7.29 or bicarbonate < 15 mmol/L. Assume 5% dehydrationwh
896
what is moderate DKA?
pH 7.1-7.19 or bicarbonate < 10 mmol/L. Assume 7% dehydration
897
what is severe DKA?
pH less than 7.1 or serum bicarbonate < 5 mmol/L. Assume 10% dehydration
898
what should be given in DKA and shock?
20 ml/kg bolus of 0.9% Sodium Chloride over 15 minutes
899
what fluid bolus should be given in DKA not in shock?
10 ml/kg 0.9% sodium chloride
900
when should the fluid bolus be subtracted from the resusitation fluids in DKA?
when not in shock
901
what fluids should be used in DKA?
0.9% sodium chloride with 20 mmol potassium chloride in each 500ml bag until glucose <14mmol/L
902
what is DiGeorge syndrome and it's features?
22q11.2 deletion congenital heart disease (e.g. tetralogy of Fallot), learning difficulties, hypocalcaemia, recurrent viral/fungal diseases, cleft palate
903
what is the name of deep rapid breathing in acidosis to rid body of CO2?
kassmaul breathing - seen in DKA
904
what is benign rolandic epilepsy?
twitching numbness or tingling episodes usually at night or when tired
904
When does neonatal hypoglycaemia require treatment?
if symptomatic or BM <1 mmol/L
905
what is the mangement of neonatal hypoglycaemia?
2.5 mg/Kg 10% dextrose
906
what is the management of foecal impaction?
1 - macrogol laxative (movicol) 2 - senna if doesnt work after 1 week use lactulose if macrogol isn't tolerate
907
what surgery is done in Hirschprungs?
Swensen's procedure
908
what is the most common cause of convergent squint?
hypermetropia (long sightedness)
909
what extra feature is present in pentology of fallot?
ASD