stuff Flashcards
1
Q
What are causes of neonatal hypotonia
A
neonatal sepsis
Werdnig-Hoffman disease (spinal muscular atrophy type 1)
hypothyroidism
Prader-Willi
maternal drugs e.g. benzodiazepines
maternal myasthenia gravis
2
Q
What is craniosynostosis
A
premature fusion of skull bones
3
Q
Limp + fever what do you do
A
admit urgently
4
Q
What is enuresis defined as
A
‘involuntary discharge of urine by day or night or both, in a child aged 5 years or older, in the absence of congenital or acquired defects of the nervous system or urinary tract’
5
Q
How do you treat infantile spasms
A
poor prognosis
vigabatrin is now considered first-line therapy
ACTH is also used
6
Q
What can cause snoring in children
A
obesity
nasal problems: polyps, deviated septum, hypertrophic nasal turbinates
recurrent tonsillitis
Down’s syndrome
hypothyroidism
7
Q
What are features of acute epiglottis
A
rapid onset
high temperature, generally unwell
stridor
drooling of saliva
‘tripod’ position: the patient finds it easier to breathe if they are leaning forward and extending their neck in a seated position
8
Q
Pansystolic murmur in lower left sternal border
A
VSD
9
Q
How do you diagnose whooping cough
A
per nasal swab culture for Bordetella pertussis - may take several days or weeks to come back
PCR and serology are now increasingly used as their availability becomes more widespread
10
Q
What is the cause of bronchioloitis
A
RSV - Respiratory syncytial virus
11
Q
How do you treat CP
A
mdt approach
spasticity - oral diazepam. oral and intrathecal baclofen
Anticonvulsants and analgesia when required
12
Q
What are features of an atypical UTI
A
Seriously ill
Poor urine flow
Abdominal or bladder mass
Raised creatinine
Septicaemia
Failure to respond to treatment with suitable antibiotics within 48 hours
Infection with non-E. coli organisms.
13
Q
How do you manage hypospadias
A
referral to specialist services
corrective surgery performed at 12 months
do not circumcise
14
Q
How do you manage ADHD
A
education/training programs
methylphenidate
15
Q
What is epsteins pearl
A
congenital cyst found in the mouth. They are common on the hard palate, but may also be seen on the gums where the parents may mistake it for an erupting tooth
16
Q
What are the tow types of innocent murmur
A
venous hums
stills murmur
17
Q
what heart condition is linked to duchennes
A
dilated cardiomyopathy
18
Q
Do you need exclusion for diarrhoea and vomiting
A
until symptoms have settled for 48 hours
19
Q
what is exomphalos (omphalocoele) and how is is treated
A
In exomphalos (also known as an omphalocoele) the abdominal contents protrude through the anterior abdominal wall but are covered in an amniotic sac formed by amniotic membrane and peritoneum.
caesarean section is indicated to reduce the risk of sac rupture
a staged repair may be undertaken as primary closure may be difficult due to lack of space/high intra-abdominal pressure
20
Q
Do you need exclusion for scabies
A
until treated
21
Q
What is the main risk factor for aspiration pneumonia
A
meconium staining
22
Q
What are features of kallmans
A
‘delayed puberty’
hypogonadism, cryptorchidism
anosmia
sex hormone levels are low
LH, FSH levels are inappropriately low/normal
patients are typically of normal or above average height
23
Q
outline paeds bls
A
unresponsive?
shout for help
open airway
look, listen, feel for breathing
give 5 rescue breaths
check for signs of circulation
infants use brachial or femoral pulse, children use femoral pulse
15 chest compressions:2 rescue breaths
chest compressions should be 100-120/min for both infants and children
depth: depress the lower half of the sternum by at least one-third of the anterior-posterior dimension of the chest (which is approximately 4 cm for an infant and 5 cm for a child)
in children: compress the lower half of the sternum
in infants: use a two-thumb encircling technique for chest compression
24
Q
How do you manage diaphragmatic hernia
A
insertion of nasogastric tube (intubation and ventilation)
surgical repair
25
What is Kochers criteria used for and what is the criteria
Non-weight bearing
Fever >38.5ºC
WCC >12 * 109/L
ESR >40mm/hr
26
What is the main difference between cephalohaematoma and caput succedaneum
C S crosses suture lines
27
What causes roseola infantum
human herpes virus 6
28
What do you see on muscle biopsy of mitochondrial diseases
red ragged fibres
29
How do you screen for DDH
breech presentation or multiple pregnancy - ultrasound - 6 weeks
newborn check and 6 week check - barlow and ortolani test
if >4.5months do xray
30
What is a contraindication for lung transplant in CF
chronic infection with Burkholderia cepacia is an important CF-specific contraindication to lung transplantation
31
What is asthma management for 5-16 year olds
1 - SABA
2 - SABA+low dose ICS
3 - SABA + paediatric low-dose ICS + leukotriene receptor antagonist (LTRA)
4 - SABA + paediatric low-dose ICS + long-acting beta agonist (LABA)
5 - SABA + switch ICS/LABA for a maintenance and reliever therapy (MART), that includes a paediatric low-dose ICS
6 - SABA + paediatric moderate-dose ICS MART
OR consider changing back to a fixed-dose of a moderate-dose ICS and a separate LABA
7 - SABA + one of the following options:
increase ICS to paediatric high-dose, either as part of a fixed-dose regime or as a MART
a trial of an additional drug (for example theophylline)
seeking advice from a healthcare professional with expertise in asthma
32
What is precocious puberty defined as
'development of secondary sexual characteristics before 8 years in females and 9 years in males'
33
How do you manage nocturnal enuresis
look for causes eg constipation, DM, UTI
general advice eg fluid
Reward system
enuresis alarm (first line)
desmopressin - short term beenfit too
34
What is the main risk factor for transient tachypnoea of the newborn
C section
35
Is whooping cough a notifiable disease
yes
36
How do you manage PDA
indomethacin
37
What are features of cystic fibrosis
neonatal period (around 20%): meconium ileus, less commonly prolonged jaundice
recurrent chest infections (40%)
malabsorption (30%): steatorrhoea, failure to thrive
other features (10%): liver disease
short stature
diabetes mellitus
delayed puberty
rectal prolapse (due to bulky stools)
nasal polyps
male infertility, female subfertility
38
what do you see on xray for NE? what is signs do you see?
dilated bowel loops (often asymmetrical in distribution)
bowel wall oedema
pneumatosis intestinalis (intramural gas)
portal venous gas
pneumoperitoneum resulting from perforation
air both inside and outside of the bowel wall (Rigler sign)
air outlining the falciform ligament (football sign)
39
What is a cephalohaematoma
swelling on the newborns head. It typically develops several hours after delivery and is due to bleeding between the periosteum and skull. The most common site affected is the parietal region
40
What are features of Turners + what is the most common renal abnormality
short stature
shield chest, widely spaced nipples
webbed neck
bicuspid aortic valve (15%), coarctation of the aorta (5-10%)
an increased risk of aortic dilatation and dissection are the most serious long-term health problems for women with Turner's syndrome
regular monitoring in adult life for these complications is an important component of care
primary amenorrhoea
cystic hygroma (often diagnosed prenatally)
high-arched palate
short fourth metacarpal
multiple pigmented naevi
lymphoedema in neonates (especially feet)
gonadotrophin levels will be elevated
hypothyroidism is much more common in Turner's
horseshoe kidney: the most common renal abnormality in Turner's syndrome
41
What are features of androgen insensitivity syndrome
'primary amenorrhoea'
undescended testes causing groin swellings
breast development may occur as a result of conversion of testosterone to oestradiol
42
what complications are there for downs
subfertility
short stature
repeated respiratory infections (+hearing impairment from glue ear)
acute lymphoblastic leukaemia
hypothyroidism
Alzheimer's disease
atlantoaxial instability
43
What are the causes of acyanotic congenital heart disease
ventricular septal defects (VSD) - most common, accounts for 30%
atrial septal defect (ASD)
patent ductus arteriosus (PDA)
coarctation of the aorta
aortic valve stenosis
44
What should you not do in acute epiglottis
Do not examine the throat
45
What are risk factors for DDH
female sex: 6 times greater risk
breech presentation
positive family history
firstborn children
oligohydramnios
birth weight > 5 kg
congenital calcaneovalgus foot deformity
46
What are features of pierre-robin syndrome
Micrognathia
Posterior displacement of the tongue (may result in upper airway obstruction)
Cleft palate
47
What are features of intestinal malrotation + how do you diagnose and treat
High caecum at the midline
Feature in exomphalos, congenital diaphragmatic hernia, intrinsic duodenal atresia
May be complicated by the development of volvulus, an infant with volvulus may have bile stained vomiting
Diagnosis is made by upper GI contrast study and USS
Treatment is by laparotomy, if volvulus is present (or at high risk of occurring then a Ladd's procedure is performed (includes division of Ladd bands and widening of the base of the mesentery)
48
What causes threadworms
Enterobius vermicularis
49
What is gastroschisis
Gastroschisis describes a congenital defect in the anterior abdominal wall just lateral to the umbilical cord
50
What is pulmonary hypoplasia and what causes it
Pulmonary hypoplasia is a term used for newborn infants with underdeveloped lungs
Causes include
oligohydramnios
congenital diaphragmatic hernia
51
What would you do if you had ITP with lymphadenopathy
bone marrow examinations is only required if there are atypical features e.g.
lymph node enlargement/splenomegaly, high/low white cells
failure to resolve/respond to treatment
52
What cardiac problems are seen in downs
multiple cardiac problems may be present
endocardial cushion defect (most common, 40%, also known as atrioventricular septal canal defects)
ventricular septal defect (c. 30%)
secundum atrial septal defect (c. 10%)
tetralogy of Fallot (c. 5%)
isolated patent ductus arteriosus (c. 5%)
53
What are features of chickenpox
fever initially
itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular
systemic upset is usually mild
54
What genetic pattern is seen in prader willi
imprinting
55
What would hemiplegic CP look like
involve one side of the patient's body
56
do you need to exclude for hand foot and mouth disease
No
57
What is laryngomalacia
Congenital abnormality of the larynx.
Infants typical present at 4 weeks of age with:
stridor
58
What is whooping cough caused by
Bordetella pertussis
59
What are features of impetigo
'golden', crusted skin lesions typically found around the mouth
very contagious
60
How do you treat pneumonia in a child
amoxicillin first line
add a macrolide if this doesnt work
use a macrolide if you suspect mycoplasma or chlamydia
if associated with influenza, use coamoxiclav
61
What is the main cause of gastroenteritis in children
rotavirus
62
what are complications of whooping cough
subconjunctival haemorrhage
pneumonia
bronchiectasis
seizures
63
What is group b strep specifically known as
Streptococcus agalactiae
64
what are features of duchenne muscular dystrophy + what sign is shown + what mode of inheritance
progressive proximal muscle weakness from 5 years
calf pseudohypertrophy
Gower's sign: child uses arms to stand up from a squatted position
30% of patients have intellectual impairment
X linked recessive
65
What is management of SUFE
internal fixation across the growth plate
66
What features do you see in 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
lumbar lordosis
67
What are high risk features sepsis
Pale/mottled/ashen/blue
No response to social cues
* Appears ill to a healthcare professional
* Does not wake or if roused does not stay awake
* Weak, high-pitched or continuous cry
Grunting
* Tachypnoea: respiratory rate >60 breaths/minute
* Moderate or severe chest indrawing
Reduced skin turgor
* Age <3 months, temperature >=38°C
* Non-blanching rash
* Bulging fontanelle
* Neck stiffness
* Status epilepticus
* Focal neurological signs
* Focal seizures
68
Is there exclusion for roseola infantum
No
69
What does VSD increase the risk of
endocarditis
70
What are features of Osgood-Schlatter disease (tibial apophysitis)
Seen in sporty teenagers
Pain, tenderness and swelling over the tibial tubercle
71
How do you manage whooping cough
<6months - admit
Notifiable disease
oral macrolide if onset of cough is in last 21 days
prophylaxis for household members
72
What needs to be monitored with methylphenidate usage
monitor ECG before starting - drugs are cardiotoxic
weight and height every 6 months
73
How do you work out apgar scores
74
When in the year is croup more common
autumn
75
how do you treat headlice
malathion, wet combing, dimeticone, isopropyl myristate and cyclomethicone
household contacts of patients with head lice do not need to be treated unless they are also affected
76
Whata re features of oesophageal atresia
Associated with tracheo-oesophageal fistula and polyhydramnios
May present with choking and cyanotic spells following aspiration
VACTERL associations
77
What are features of rickets
aching bones and joints
lower limb abnormalities:
in toddlers genu varum (bow legs)
in older children - genu valgum (knock knees)
'rickety rosary' - swelling at the costochondral junction
kyphoscoliosis
craniotabes - soft skull bones in early life
Harrison's sulcus
78
What LH and testosterone do you see in Klinefelters, Kallmans, androgen insensitivity syndrome and a testerone secreting tumour
79
What is meant by genetic anticipation
earlier onset in successive generations
80
Where does bile enter the GI tract (and therefore when bilious vomiting occurs)
second part of duodenum
81
What are features of measles
prodromal phase
irritable
conjunctivitis
fever
Koplik spots
typically develop before the rash
white spots ('grain of salt') on the buccal mucosa
rash
starts behind ears then to the whole body
discrete maculopapular rash becoming blotchy & confluent
desquamation that typically spares the palms and soles may occur after a week
diarrhoea occurs in around 10% of patients
82
management of neonatal hypoglycaemia
asymptomatic
encourage normal feeding (breast or bottle)
monitor blood glucose
symptomatic or very low blood glucose
admit to the neonatal unit
intravenous infusion of 10% dextrose
83
What are features of Mumps
Fever, malaise, muscular pain
Parotitis ('earache', 'pain on eating'): unilateral initially then becomes bilateral in 70%
84
Asthma management in kid <5 years
1 - Short-acting beta agonist (SABA)
2 - SABA + an 8-week trial of paediatric MODERATE-dose inhaled corticosteroid (ICS)
After 8-weeks stop the ICS and monitor the child's symptoms:
if symptoms did not resolve during the trial period, review whether an alternative diagnosis is likely
if symptoms resolved then reoccurred within 4 weeks of stopping ICS treatment, restart the ICS at a paediatric low dose as first-line maintenance therapy
if symptoms resolved but reoccurred beyond 4 weeks after stopping ICS treatment, repeat the 8-week trial of a paediatric moderate dose of ICS
3 - SABA + paediatric low-dose ICS + leukotriene receptor antagonist (LTRA)
4 - Stop the LTRA and refer to an paediatric asthma specialist
85
if neonatal hypoglycaemia is <1mmol what do you do
IV 10% dextrose
86
what is ophthalmia neonatum
infection of the eye
usually conjunctivitis - discharge
87
What are characteristics of an innocent murmur
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
88
what do you see on xray with TTN
heart failure type pattern (e.g. interstitial oedema and pleural effusions) but key distinguishing features from congenital heart disease are a normal heart size and rapid resolution of the failure type pattern within days.
89
What are the causes of precocious puberty in males
Testes
bilateral enlargement = gonadotrophin release from intracranial lesion
unilateral enlargement = gonadal tumour
small testes = adrenal cause (tumour or adrenal hyperplasia)
90
How do you manage eczema
avoid irritants
simple emolients
topical steroids
wet wrapping
oral ciclosporin in severe cases
91
What are features of downs syndrome
face: upslanting palpebral fissures, epicanthic folds, Brushfield spots in iris, protruding tongue, small low-set ears, round/flat face
flat occiput
single palmar crease, pronounced 'sandal gap' between big and first toe
hypotonia
congenital heart defects (40-50%, see below)
duodenal atresia
Hirschsprung's disease
92
What are features of Scarlet Fever + what causes it
Reaction to erythrogenic toxins produced by Group A haemolytic streptococci
Fever, malaise, tonsillitis
'Strawberry' tongue
Rash - fine punctate erythema sparing the area around the mouth (circumoral pallor)
93
What do you do with acute epiglottis when medical therapy is not enough
endotracheal intubation
94
How do you investigate meckels diverticulum
if the child is haemodynamically stable with less severe or intermittent bleeding then a 'Meckel's scan' should be considered
uses 99m technetium pertechnetate, which has an affinity for gastric mucosa
mesenteric arteriography may also be used in more severe cases e.g. transfusion is required
95
How do you treat NE
laporotomy
96
investigation for NE
abdominal xray
97
What is Meckels diverticulum
congenital diverticulum of the small intestine. It is a remnant of the omphalomesenteric duct (also called the vitellointestinal duct) and contains ectopic ileal, gastric or pancreatic mucosa.
98
What is management for constipation
polyethylene glycol 3350 + electrolytes (Movicol Paediatric Plain)
add a stimulant laxative if Movicol Paediatric Plain does not lead to disimpaction after 2 weeks
maintenance therapy:
first-line: Movicol Paediatric Plain
add a stimulant laxative if no response
99
What do you see on EEG in benign rolandic epilepsy
centrotemporal strikes
100
What are the differences between early (compensated) shock and late (decompensated) shock
101
What is the cause of croup
parainfluenza virus
102
What age does bronchiolitis typically impact
<1-2 year
103
what staging system is used for perthes
Catterall staging
104
What causes homocystinuria
deficiency of cystathionine beta synthase
105
What signs are there for biliary atresia
Jaundice
Hepatomegaly with splenomegaly
Abnormal growth
Cardiac murmurs if associated cardiac abnormalities present
106
how is pyloric stenosis managed
Ramstedt pyloromyotomy.
107
What is the criteria for an immediate CT if a child has hit their head
Loss of consciousness lasting more than 5 minutes (witnessed)
Amnesia (antegrade or retrograde) lasting more than 5 minutes
Abnormal drowsiness
Three or more discrete episodes of vomiting
Clinical suspicion of non-accidental injury
Post-traumatic seizure but no history of epilepsy
GCS less than 14, or for a baby under 1 year GCS (paediatric) less than 15, on assessment in the emergency department
Suspicion of open or depressed skull injury or tense fontanelle
Any sign of basal skull fracture (haemotympanum, panda' eyes, cerebrospinal fluid leakage from the ear or nose, Battle's sign)
Focal neurological deficit
If under 1 year, presence of bruise, swelling or laceration of more than 5 cm on the head
Dangerous mechanism of injury (high-speed road traffic accident either as pedestrian, cyclist or vehicle occupant, fall from a height of greater than 3 m, high-speed injury from a projectile or an object)
108
How do you investigate \hirschprungs
rectal biopsy
109
What are features of Rubella
Rash: pink maculopapular, initially on face before spreading to whole body, usually fades by the 3-5 day
Lymphadenopathy: suboccipital and postauricular
110
What are features of Dyskinetic CP
caused by damage to the basal ganglia and the substantia nigra
athetoid movements and oro-motor problems
involuntary movements, commonly affecting the hands and feet. The excessive salivation and mastication difficulties observed are indicative of compromised control over facial muscles
111
What classes severity of alpha thalassemia and what are typical findings
If 1 or 2 alpha globulin alleles are affected then the blood picture would be hypochromic and microcytic, but the Hb level would be typically normal
If are 3 alpha globulin alleles are affected results in a hypochromic microcytic anaemia with splenomegaly. This is known as Hb H disease
If all 4 alpha globulin alleles are affected (i.e. homozygote) then death in utero (hydrops fetalis, Bart's hydrops)
112
What are features of osteochondritis dissecans
Pain after exercise
Intermittent swelling and locking
113
What are features of Patellar tendonitis
More common in athletic teenage boys
Chronic anterior knee pain that worsens after running
Tender below the patella on examination
114
What are features of hand foot and mouth disease
mild systemic upset: sore throat, fever
oral ulcers
followed later by vesicles on the palms and soles of the feet
115
What causes impetigo
staph/strep
116
How is pyloric stenosis diagnosed
ultrasound
117
Which disorders show genetic anticipation
Trinucleotide repeat disorders:
Fragile X (CGG)
Huntington's (CAG)
myotonic dystrophy (CTG)
Friedreich's ataxia* (GAA)
spinocerebellar ataxia
spinobulbar muscular atrophy
dentatorubral pallidoluysian atrophy
*Friedreich's ataxia is unusual in not demonstrating anticipation
118
What chromosome trisomies are linked to congenital disorders eg downs
patau 13
edwards 18
downs 21
119
What are features of reflex anoxic seizures
child goes very pale
falls to floor
secondary anoxic seizures are common
rapid recovery
120
How do you grade VUR
121
How do you manage perthes
To keep the femoral head within the acetabulum: cast, braces
If less than 6 years: observation
Older: surgical management with moderate results
Operate on severe deformities
122
123
How do you manage meckels diverticulum
Management
removal if narrow neck or symptomatic
options are between wedge excision or formal small bowel resection and anastomosis
124
What are features of JIA
joint pain and swelling: usually medium sized joints e.g. knees, ankles, elbows
limp
ANA may be positive in JIA - associated with anterior uveitis
125
What are features of edwards syndrome
Micrognathia
Low-set ears
Rocker bottom feet
Overlapping of fingers
126
What is headlice caused by
Pediculus capitis
127
What is Vesicoureteric reflux
abnormal backflow of urine from the bladder into the ureter and kidney. It is a relatively common abnormality of the urinary tract in children and predisposes to urinary tract infection (UTI)
128
What is the barlow and ortolani test
Barlow test: attempts to dislocate an articulated femoral head
Ortolani test: attempts to relocate a dislocated femoral head
129
How do threadworms manifest
perianal itching, particularly at night
girls may have vulval symptoms
130
How do you manage ophthalmia neonatum
refer same day
131
What are features of TGA + what sign + what heart sound
cyanosis
tachypnoea
loud single S2
prominent right ventricular impulse
'egg-on-side' appearance on chest x-ray
132
When are you infectious with chickenpox
Advise that the most infectious period is 1-2 days before the rash appears, but infectivity continues until all the lesions are dry and have crusted over (usually about 5 days after the onset of the rash).
133
what are features of ITP
bruising
petechial or purpuric rash
bleeding is less common and typically presents as epistaxis or gingival bleeding
134
What are features of whooping cough
catarrhal phase
symptoms are similar to a viral upper respiratory tract infection
lasts around 1-2 weeks
paroxysmal phase
the cough increases in severity
coughing bouts are usually worse at night and after feeding, may be ended by vomiting & associated central cyanosis
inspiratory whoop: not always present (caused by forced inspiration against a closed glottis)
infants may have spells of apnoea
persistent coughing may cause subconjunctival haemorrhages or even anoxia leading to syncope & seizures
lasts between 2-8 weeks
convalescent phase
the cough subsides over weeks to months
135
What are risk factors for neonatal sepsis
Mother who has had a previous baby with GBS infection, who has current GBS colonisation from prenatal screening, current bacteruria, intrapartum temperature ≥38ºC, membrane rupture ≥18 hours, or current infection throughout pregnancy
Premature (<37 weeks): approximately 85% of neonatal sepsis cases are in premature neonates
Low birth weight (<2.5kg): approximately 80% are low birth weight
Evidence of maternal chorioamnionitis
136
What are the most common fractures associated with child abuse
- Radial
- Humeral
- Femoral
137
What causes ophthalmia neonatum
chlamydia
gonorrhoea
138
What are features of fragile x
Learning difficulties
Macrocephaly
Long face
Large ears
Macro-orchidism
139
What is ebsteins anomaly + what features + heart defects
caused by the use of lithium in pregnancy. It occurs when the posterior leaflets of the tricuspid valve are displaced anteriorly towards the apex of the right ventricle. The creates tricuspid regurgitation (pan-systolic murmur) and tricuspid stenosis (mid-diastolic murmur). There is also enlargement of the right atrium.
cyanosis
prominent 'a' wave in the distended jugular venous pulse,
hepatomegaly
tricuspid regurgitation
pansystolic murmur, worse on inspiration
right bundle branch block → widely split S1 and S2
140
What are features of intussusception
intermittent, severe, crampy, progressive abdominal pain
inconsolable crying
during paroxysm the infant will characteristically draw their knees up and turn pale
vomiting
bloodstained stool - 'red-currant jelly' - is a late sign
sausage-shaped mass in the right upper quadrant
141
What can you give to help with recurrent febrile convulsions
rectal diazepam or buccal midazolam
142
How do you investigate duchennes
raised creatinine kinase
genetic testing has now replaced muscle biopsy as the way to obtain a definitive diagnosis
143
What time intervals do you measure apgar score
1,5 minutes,
if low, repeat every 10
144
What is infantile colic
Excessive crying and pulling up of legs, often worse in evenings
Reassurance and support
<3 months old
145
What are features of lymphatic malformations
Usually located posterior to the sternocleidomastoid
Cystic hygroma result from occlusion of lymphatic channels
The painless, fluid filled, lesions usually present prior to the age of 2
They are often closely linked to surrounding structures and surgical removal is difficult
They are typically hypoechoic on USS
146
When should a PPI be used in GORD
PPI should be trialled in infants with GORD who do not respond to alginates/thickened feeds and who have 1. feeding difficulties, 2. distressed behaviour or 3. faltering growth
147
What investigations do you do for infantile spasms + what do you see
the EEG shows hypsarrhythmia in two-thirds of infants
CT demonstrates diffuse or localised brain disease in 70% (e.g. tuberous sclerosis)
148
limp <3 years old
admit
149
When does immune thrombocytopenia present
after infection/vaccine
150
how do you manage ddh
pavlik harness if <4/5 months
151
What mode of inheritance is achondroplasia + what is mutation
autosomal dominant
mutation in FGFR-3 gene
152
What are life threatening features of asthma attack
SpO2 <92%
PEF <33% best or predicted
Silent chest
Poor respiratory effort
Agitation
Altered consciousness
Cyanosis
153
What would monoplegic CP look like
impacts a single limb
154
Diastolic machinery murmur in the upper left sternal border
PDA
155
How do you manage NRDS
prevention during pregnancy: maternal corticosteroids to induce fetal lung maturation
oxygen
assisted ventilation
exogenous surfactant given via endotracheal tube
156
What causes Scarlet Fever
Group A haemolytic streptococci
157
What are features of a wilms tumour
abdominal mass (most common presenting feature)
painless haematuria
flank pain
other features: anorexia, fever
unilateral in 95% of cases
metastases are found in 20% of patients (most commonly lung)
158
How do you manage meningitis
< 3 months: IV amoxicillin (or ampicillin) + IV cefotaxime
> 3 months: IV cefotaxime (or ceftriaxone)
159
What is plagiocephaly
parallelogram shaped head
the incidence of plagiocephaly has increased over the past decade. This may be due to the success of the 'Back to Sleep' campaign
160
When is heel prick test done
5-9 days
161
What are features of Erythema infectiosum (Slapped cheek syndrome) + what causes it
Also known as fifth disease or 'slapped-cheek syndrome'
Caused by parvovirus B19
Lethargy, fever, headache
'Slapped-cheek' rash spreading to proximal arms and extensor surfaces
162
Crescendo-decrescendo murmur in the upper left sternal border
coarctation of the aorta
163
At what age do you typically get intussusception
6-18 months
164
Where does atopic eczema typically effect infants
nappy area and trunk
165
what is the first sign of puberty in women
breast development
166
How do you manage CMPI/A
if formula fed:
extensive hydrolysed formula (eHF) milk is the first-line replacement formula for infants with mild-moderate symptoms
amino acid-based formula (AAF) in infants with severe CMPA or if no response to eHF
if breastfeeding:
continue breastfeeding
eliminate cow's milk protein from maternal diet. Consider prescribing calcium supplements for breastfeeding mothers
use eHF milk when breastfeeding stops, until 12 months of age and at least for 6 months
167
When does infantile colic typically stop
6 months
168
What are causes of jaundice in first 24 hours
PATHOLOGICAL
rhesus haemolytic disease
ABO haemolytic disease
hereditary spherocytosis
glucose-6-phosphodehydrogenase
169
What are features of noonan syndrome
Webbed neck
Pectus excavatum
Short stature
Pulmonary stenosis
170
Where are the alpha globulin genes in alpha thalaessemia found
ch16
171
What is perthes disease
degenerative condition affecting the hip joints of children, typically between the ages of 4-8 years. It is due to avascular necrosis of the femoral head, specifically the femoral epiphysis. Impaired blood supply to the femoral head causes bone infarction.
172
What are cardiac defects associated with Turners syndrome
Bicuspid aortic valve (most common)
Aortic root dilatation
Coarctation of the aorta
173
What are features of patella subluxation
Medial knee pain due to lateral subluxation of the patella
Knee may give way
174
What do you see on imaging of intussusception
abdominal film xray - sausage shaped mass
ultrasound - target sign
175
What are features of Cri du chat syndrome (chromosome 5p deletion syndrome)
Characteristic cry (hence the name) due to larynx and neurological problems
Feeding difficulties and poor weight gain
Learning difficulties
Microcephaly and micrognathism
Hypertelorism
176
in peads bls, what ratio of compressions do you do
15:2 if there are 2 people, one LAY person - 30:2
177
What do you see on xray for croup
a posterior-anterior view will show subglottic narrowing, commonly called the 'steeple sign'
in contrast, a lateral view in acute epiglottis will show swelling of the epiglottis - the 'thumb sign'
178
what is a venous hum
Due to the turbulent blood flow in the great veins returning to the heart. Heard as a continuous blowing noise heard just below the clavicles
179
What are complications of undescended testes
infertility
torsion
testicular cancer
psychological
180
what is a reflex anoxic seizure
a syncopal episode (or presyncope) that occurs in response to pain or emotional stimuli
181
What causes Erythema infectiosum
Parvovirus B19
182
What are features of prader willi
hypotonia during infancy
dysmorphic features
short stature
hypogonadism and infertility
learning difficulties
childhood obesity
behavioural problems in adolescence
willi - willy - floppy
183
When should you admit for croup
<3months
moderate or severe croup
known upper airway abnormalities (e.g. Laryngomalacia, Down's syndrome)
uncertainty about diagnosis (important differentials include acute epiglottitis, bacterial tracheitis, peritonsillar abscess and foreign body inhalation)
184
What are complications of measles
otitis media: the most common complication
pneumonia: the most common cause of death
encephalitis: typically occurs 1-2 weeks following the onset of the illness)
subacute sclerosing panencephalitis: very rare, may present 5-10 years following the illness
febrile convulsions
keratoconjunctivitis, corneal ulceration
diarrhoea
increased incidence of appendicitis
myocarditis
185
how does biliary atresia present
FIRST FEW WEEKS OF LIFE
Jaundice extending beyond the physiological two weeks
Dark urine and pale stools
Appetite and growth disturbance, however, may be normal in some cases
186
What is management of chickenpox
calamine lotion
school exclusion: NICE Clinical Knowledge
187
How do you manage acute epiglottis
Senior involvement - emergency
Intubate if necessary
oxygen if needed
IV ABx
188
Typical paediatric vitals (HR and RR)
189
What should you do if a neonate presents with jaundice
measure serum bilirubin urgently
190
How is JIA defined
occurring in someone who is less than 16 years old that lasts for more than 6 weeks. Pauciarticular JIA refers to cases where 4 or less joints are affected. It accounts for around 60% of cases of JIA
191
What is a common deficiency in newborns
vit k
can lead to haemorrhagic disease
antiepileptics increases risk
offer vit k orally or IM
192
What incerases risk of having a child with TGA
diabetets
193
How do you diagnose CF
immue reactive trypsinogen
sweat testW
194
What is the first sign of puberty in males
testicular growth
195
What is the difference of timing between NRDS and TTN
Neonates with NRDS usually present with respiratory distress shortly after birth which usually worsens over the next few days. In contrast, TTN usually presents with tachypnoea shortly after birth and often fully resolves within the first day of life
196
What is the main riks factor for neonatal respiratory distress syndrome
prematurity
197
What are features of Measles
Prodrome: irritable, conjunctivitis, fever
Koplik spots: white spots ('grain of salt') on buccal mucosa
Rash: starts behind ears then to whole body, discrete maculopapular rash becoming blotchy & confluent
198
What are features of infantile haemangioma
May present in either triangle of the neck
Grow rapidly initially and then will often spontaneously regress
Plain x-rays will show a mass lesion, usually containing calcified phleboliths
As involution occurs the fat content of the lesions increases
199
How do you diagnose vesicoureteric reflux
micturating cystourethrogram
200
What are the features of TOF + what murmur + what on xray
ventricular septal defect (VSD)
right ventricular hypertrophy
right ventricular outflow tract obstruction, pulmonary stenosis
overriding aorta
cyanosis
unrepaired TOF infants may develop episodic hypercyanotic 'tet' spells due to near occlusion of the right ventricular outflow tract
features of tet spells include tachypnoea and severe cyanosis that may occasionally result in loss of consciousness
they typically occur when an infant is upset, is in pain or has a fever
**causes a right-to-left shunt**
**ejection systolic murmur** due to pulmonary stenosis (the VSD doesn't usually cause a murmur)
a right-sided aortic arch is seen in 25% of patients
chest x-ray shows a '**boot-shaped**' heart, ECG shows right ventricular hypertrophy
201
what is a stills murmur
Low-pitched sound heard at the lower left sternal edge
202
What are features of febrile convulsion
usually occur early in a viral infection as the temperature rises rapidly
seizures are usually brief, lasting less than 5 minutes
are most commonly tonic-clonic
203
How do you manage impetigo
hydrogen peroxide cream 1%
204
what can chickenpox + group A strep result in
necrotizing fasciitis
205
What are features of patau syndrome
Microcephalic, small eyes
Cleft lip/palate
Polydactyly
Scalp lesions
206
How is acute epiglottis diagnosed
Diagnosis is made by direct visualisation (only by senior/airway trained staff, see below). However, x-rays may be done
207
What are features of williams syndrome + what heart condition
short stature
Learning difficulties
Friendly, extrovert personality
Transient neonatal hypercalcaemia
Supravalvular aortic stenosis
elfin facies
208
What are contraindications to LP in suspected meningitis
focal neurological signs
papilloedema
significant bulging of the fontanelle
disseminated intravascular coagulation
signs of cerebral herniation
209
what is the most common cause of ambiguous genitalia
congenital adrenal hyperplasia
210
How do you manage scarlet fever
oral penicillin V (phenoxymethylpenicillin) 10 days
azithromycin if allergy
211
What are causes of prolonged jaundice
biliary atresia
hypothyroidism
galactosaemia
urinary tract infection
breast milk jaundice
jaundice is more common in breastfed babies
prematurity
due to immature liver function
increased risk of kernicterus
congenital infections e.g. CMV, toxoplasmosis
212
When do you offer the whooping cough vaccine in pregnancy
16-32 weeks
213
What are features of infantile spasms
characteristic 'salaam' attacks: flexion of the head, trunk and arms followed by extension of the arms
this lasts only 1-2 seconds but may be repeated up to 50 times
progressive mental handicap
214
risk factors for neonatal hypoglycaemia
preterm birth (< 37 weeks)
maternal diabetes mellitus
IUGR
hypothermia
neonatal sepsis
inborn errors of metabolism
nesidioblastosis
Beckwith-Wiedemann syndrome
215
How do you treat intestnal malrotation
Ladds procedure
216
When are will children with non-IgE mediated intolerance become tolerant
3 years
217
How are immunisations and developmental stages affected by prematurity
dont adjust immunisations
adjust developmental - add difference from week 40
218
What is mesenteric adenitis
similar presntation to appendicitis but follows after infection
219
What classes as neonatal hypoglycaemia
<2.6
220
What are features of perthes disease
hip pain: develops progressively over a few weeks
limp
stiffness and reduced range of hip movement
x-ray: early changes include widening of joint space, later changes include decreased femoral head size/flattening
221
Is there exclusion for whooping cough
school exclusion: 48 hours after commencing antibiotics (or 21 days from onset of symptoms if no antibiotics )
222
How do you manage a wilms tumour
nephrectomy
chemotherapy
radiotherapy if advanced disease
223
do you need exclusion for influenza
until recovered
224
how do you manage intussuscpetion
reduction by air insufflation
if this fails, or signs of peritonitis -> surgery
225
What are features of GORD
typically develops before 8 weeks
vomiting/regurgitation
milky vomits after feeds
may occur after being laid flat
excessive crying, especially while feeding
226
hypoglycaemia seen hours after birth
Transient hypoglycaemia in the first hours after birth is common.
227
What does maternal antiepleptic use increase chances of
orofacial defects eg cleft palate
228
How do you manage gastroschisis
vaginal delivery may be attempted
newborns should go to theatre as soon as possible after delivery, e.g. within 4 hours
229
What are the criteria of severity for croup
230
How do you diagnose headlice
fine-toothed combing of wet or dry hair
231
When do febrile convulsions occur
6months-3years
232
What increases the chances of secondary bacterial infection with chickenpox
nsaids
233
What are features of eczema
itchy, erythematous rash
repeated scratching may exacerbate affected areas
in infants the face and trunk are often affected
in younger children, eczema often occurs on the extensor surfaces
in older children, a more typical distribution is seen, with flexor surfaces affected and the creases of the face and neck
234
ejection systolic murmur and fixed splitting of the second heart sound
ASD
235
What are features of klinefelters
often taller than average
lack of secondary sexual characteristics
small, firm testes
infertile
gynaecomastia - increased incidence of breast cancer
elevated gonadotrophin levels
236
What are the types of biliary atresia
Type 1: The proximal ducts are patent, however, the common duct is obliterated
Type 2: There is atresia of the cystic duct and cystic structures are found in the porta hepatis
Type 3: There is atresia of the left and right ducts to the level of the porta hepatis, this occurs in >90% of cases of biliary atresia
237
Do you need exclusion for impetigo
Until lesions are crusted and healed, or 48 hours
after commencing antibiotic treatment
238
How do you manage CF + what drugs
twice daily chest physio and postural drainage
high calorie high fat diet
minimise contact with other CF
vitamin supplements
pancreatic enzyme supplements with meals
Lumacaftor/Ivacaftor (Orkambi)
is used to treat cystic fibrosis patients who are homozygous for the delta F508 mutation
lumacaftor increases the number of CFTR proteins that are transported to the cell surface
ivacaftor is a potentiator of CFTR that is already at the cell surface, increasing the probability that the defective channel will be open and allow chloride ions to pass through the channel pore
239
What are features of Kawasaki disease
high-grade fever which lasts for > 5 days. Fever is characteristically resistant to antipyretics
conjunctival injection
bright red, cracked lips
strawberry tongue
cervical lymphadenopathy
red palms of the hands and the soles of the feet which later peel
240
When would you consider pneumonia in a bronchiolitis picture
fever >39
persistently focal crackles
241
What are features of pyloric stenosis
'projectile' vomiting, typically 30 minutes after a feed
constipation and dehydration may also be present
a palpable mass may be present in the upper abdomen
hypochloraemic, hypokalaemic alkalosis due to persistent vomiting
242
management for hirschprungs
rectal washouts/bowel irrigation initially
surgery to affected segment of colon
243
what are features of necrotising enterocolitis
feeding intolerance, abdominal distension and bloody stools, which can quickly progress to abdominal discolouration, perforation and peritonitis.
244
How do you manage umbilical hernia
Usually self-resolve, but if large (>1.5cm) or symptomatic perform elective repair at 2-3 years of age. If small and asymptomatic perform elective repair at 4-5 years of age.
245
How do you look at scarring for vesicoureteric reflux
DMSA scan
246
What murmur is linked to turners
ejection systolic
247
What are features of croup
cough
barking, seal-like
worse at night
stridor
remember, the throat should be not examined due to the risk of precipitating airway obstruction
fever
coryzal symptoms
increased work of breathing e.g. retraction
248
When is bowed legs in a child normal
<3 years
249
what are features of meckels diverticulum
abdominal pain mimicking appendicitis
rectal bleeding
Meckel's diverticulum is the most common cause of painless massive GI bleeding requiring a transfusion in children between the ages of 1 and 2 years
intestinal obstruction
secondary to an omphalomesenteric band (most commonly), volvulus and intussusception
250
What emergency teratment is there for croup
high-flow oxygen
nebulised adrenaline
251
What is the triad of shaken baby syndrome
Retinal haemorrhages, subdural haematoma and encephalopathy
252
What can cause a false positive sweat test
malnutrition
adrenal insufficiency
glycogen storage diseases
nephrogenic diabetes insipidus
hypothyroidism, hypoparathyroidism
G6PD
ectodermal dysplasia
253
outline newborn resuscitation
1. Dry baby and maintain temperature
2. Assess tone, respiratory rate, heart rate
3. If gasping or not breathing give 5 inflation breaths*
4. Reassess (chest movements)
5. If the heart rate is not improving and <60bpm start compressions and ventilation breaths at a rate of 3:1
254
how do you manage croup
single dose of oral dexamethasone (0.15mg/kg) to all children regardless of severity
255
What is acute epiglottis caused by
Haemophilus influenzae type B
256
How do you manage threadworms
hygiene measures for everyone in house
single dose mebendazole is used first-line for children > 6 months old and to household
257
What are features of a brachial cyst
Six branchial arches separated by branchial clefts
Incomplete obliteration of the branchial apparatus may result in cysts, sinuses or fistulae
75% of branchial cysts originate from the second branchial cleft
Usually located anterior to the sternocleidomastoid near the angle of the mandible
Unless infected the fluid of the cyst has a similar consistency to water and is anechoic on USS
258
What are features of sexual abuse
pregnancy
sexually transmitted infections, recurrent UTIs
sexually precocious behaviour
anal fissure, bruising
reflex anal dilatation
enuresis and encopresis
behavioural problems, self-harm
recurrent symptoms e.g. headaches, abdominal pain
259
What do you do if a hernia incarcerates
it should be manually reduced with pressure and surgically repaired within 24 hours. If it can't be reduced, an emergency operation is required. His hernia has not incarcerated and hence does not require an emergency operation.
260
What is the difference between Cows milk protein intolerance and allergy
Both immediate (IgE mediated) and delayed (non-IgE mediated) reactions are seen. The term CMPA is usually used for immediate reactions and CMPI for mild-moderate delayed reactions.
261
What genetic condition is associated with intussusception
downs
262
What anatomical changes do you see in TGA
aorta leaves the right ventricle
pulmonary trunk leaves the left ventricle
263
What are features of spastic CP
subtypes include hemiplegia, diplegia or quadriplegia
increased tone resulting from damage to upper motor neurons in periventricular white matter
hypertonia resulting in muscular stiffness
264
What is the main complication of scarlet fever
otitis media
can cause invasive features eg bacteraemia or necrotising fasciitis
265
What are the 3 types of cerebral palsy
Spastic 70%
Dyskinetic
Ataxic
Mixed
266
What is management of neonatal sepsis
IV benzylpenicillin + gentamicin
267
What are symptoms of hypernatraemia due to dehydration
jittery movements
increased muscle tone
hyperreflexia
convulsions
drowsiness or coma
268
What is CPAP used in
sleep apnoea
269
What conditions are associated with hypospadias
cryptorchidism
inguinal hernia
270
How do you manage undescended testes + what is the name of the procedure
unilateral - refer at 3 months, ideally have a surgeon by 6 months Orchidopexy
bilateral - urgent review within 24 hours
271
How does perthes usually present
Hip pain (may be referred to the knee) usually occurring between 5 and 12 years of age. Bilateral disease in 20%.
272
What do you see on xray with NRDS
diffuse ground glass lungs with low volumes and a bell-shaped thorax
indistinct heart border
273
What do you see on xray of acute epiglottis
a lateral view in acute epiglottis will show swelling of the epiglottis - the 'thumb sign'
in contrast, a posterior-anterior view in croup will show subglottic narrowing, commonly called the 'steeple sign'
274
What is benign rolandic epilepsy
occurs between 4-12
seizures characteristically occur at night
seizures are typically partial (e.g. paraesthesia affecting the face) but secondary generalisation may occur (i.e. parents may only report tonic-clonic movements)
the child is otherwise normal
275
What hearing tests are done for kids and when
Newborn - otoacoustic emission test -> auditory brainstem response if abnormal
6-9 months - distraction test
18months-2.5years - recognition of familiar objects
>2.5 years - perforamnce test and speech discrimination tests
>3 years - Pure tone audiometry - done at school
276
What causes Hand foot and mouth disease
Coxsackie A16 virus
or enterovirus 71
277
What are causes of obesity in children
growth hormone deficiency
hypothyroidism
Down's syndrome
Cushing's syndrome
Prader-Willi syndrome
278
What is diagnostic criteria for whooping cough
Whooping cough should be suspected if a person has an acute cough that has lasted for 14 days or more without another apparent cause, and has one or more of the following features:
Paroxysmal cough.
Inspiratory whoop.
Post-tussive vomiting.
Undiagnosed apnoeic attacks in young infants.
279
What are features of roseola infantum and what complication is there
high fever: lasting a few days, followed later by a
maculopapular rash
Nagayama spots: papular enanthem on the uvula and soft palate
febrile convulsions occur in around 10-15%
diarrhoea and cough are also commonly seen
280
What are features of fragile x
learning difficulties
large low set ears, long thin face, high arched palate
macroorchidism
hypotonia
autism is more common
mitral valve prolapse
281
What type of vaccine is the rotavirus
oral live attenuated vaccine
282
What significant complication is associated with kawasaki disease
coronary artery aneurysms
do echo
283
Do you need exclusion for slapped cheek
no
284
What is the most common cause of pneumonia in children
strep pne
285
What are features of retinoblastoma + what is mode of inheritance
autosomal dominant
most common ocular malignancy in children
absence of red-reflex, replaced by a white pupil (leukocoria) - the most common presenting symptom
strabismus
visual problems
286
What is the most common cyanotic congenital heart disease
TOF, but TGA is more common at birth
287
How does SUFE present
Typically seen in obese male adolescents. Pain is often referred to the knee. Limitation to internal rotation is usually seen. Knee pain is usually present 2 months prior to hip slipping. Bilateral in 20%.
288
What types of febrile convulsion can you get and what are features
289
What shoudl you do with someone <6months with atypical UTI
ultrasound
290
features of neonatal hypoglycaemia
autonomic (hypoglycaemia → changes in neural sympathetic discharge)
'jitteriness'
irritable
tachypnoea
pallor
neuroglycopenic
poor feeding/sucking
weak cry
drowsy
hypotonia
seizures
other features may include
apnoea
hypothermia
291
What are features of Hand, Foot and Mouth disease
Caused by the coxsackie A16 virus
Mild systemic upset: sore throat, fever
Vesicles in the mouth and on the palms and soles of the feet
292
What are features of foetal alcohol syndrome
Baby may show symptoms of alcohol withdrawal at birth e.g. irritable, hypotonic, tremors
Features
short palpebral fissure
thin vermillion border/hypoplastic upper lip
smooth/absent filtrum
learning difficulties
microcephaly
growth retardation
epicanthic folds
cardiac malformations
293
How do you manage biliary atresia
Surgical intervention is the only definitive treatment for biliary atresia: Intervention may include dissection of the abnormalities into distinct ducts and anastomosis creation
Medical intervention includes antibiotic coverage and bile acid enhancers following surgery
294
How do you diagnose perthes
x ray
295
how do you manage acute asthma
Bronchodilator therapy
give a beta-2 agonist via a spacer (for a child < 3 years use a close-fitting mask)
give 1 puff every 30-60 seconds up to a maximum of 10 puffs
if symptoms are not controlled repeat beta-2 agonist and refer to hospital
Steroid therapy
should be given to all children with an asthma exacerbation
treatment should be given for 3-5 days
296
Ejection systolic murmur in the upper left sternal border
pulmonary stenosis
297
How do you manage GORD
advice - positioning (30 degrees up), dont overfeed, sleep on back
trial of thickened formula (if bottle fed)
trial of alginate therapy eg gaviscon (dont use at same time as thickened formula)
298
What are features of chickenpox
Fever initially
Itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular
Systemic upset is usually mild
299
Why do you not give aspirin to children
Reye syndrome
300
Do you need exclusion for infectious mononucleosis
no
301
What investigation results do you see with biliary atresia
Total bilirubin may be normal, whereas conjugated bilirubin is abnormally high
302
what determines the degree of cyanosis and severity in TOF
The severity of the right ventricular outflow tract obstruction
303
What are features of dermoid cysts
Derived from pleuripotent stem cells and are located in the midline
Most commonly in a suprahyoid location
They have heterogeneous appearances on imaging and contain variable amounts of calcium and fat
304
What is the most common cause of hypothyroidism in kids
autoimmune thyroiditis
305
Do you need exclusion for rubella
5 days after rash
306
What are features of a thyroglossal cyst
Located in the anterior triangle, usually in the midline and below the hyoid (65% cases)
Derived from remnants of the thyroglossal duct
Thin walled and anechoic on USS (echogenicity suggests infection of cyst)
307
What is hypospadias
a ventral urethral meatus
a hooded prepuce
chordee (ventral curvature of the penis) in more severe forms
the urethral meatus may open more proximally in the more severe variants. However, 75% of the openings are distally located.
308
How do you treat biliary atresia
Kasai procedure
309
What are features of CMPI/A
regurgitation and vomiting
diarrhoea
urticaria, atopic eczema
'colic' symptoms: irritability, crying
wheeze, chronic cough
rarely angioedema and anaphylaxis may occur
310
What are features of McCune-Albright syndrome
somatic mutation in the GNAS gene.
Features
precocious puberty
cafe-au-lait spots
polyostotic fibrous dysplasia
short stature
311
how do you investigate hirschsprungs
rectal biopsy
312
Do you need exclusion for mumps
5 days after onset of swollen glands
313
How do you manage kawasaki
high dose aspirin
IV IG
echo
314
What are features of PDA
left subclavicular thrill
continuous 'machinery' murmur
large volume, bounding, collapsing pulse
wide pulse pressure
heaving apex beat
315
What are features of prader willi syndrome
Hypotonia
Hypogonadism
Obesity
316
What are features of chondromalacia patellae
Softening of the cartilage of the patella
Common in teenage girls
Characteristically anterior knee pain on walking up and down stairs and rising from prolonged sitting
Usually responds to physiotherapy
317
What is patent ductus arteriosus
connection between the pulmonary trunk and descending aorta
usually, the ductus arteriosus closes with the first breaths due to increased pulmonary flow which enhances prostaglandins clearance
more common in premature babies, born at high altitude or maternal rubella infection in the first trimester
318
What investigation do you do for intussusception
ultrasound
319
What are risk factors for sudden infant death syndrome
prone sleeping
parental smoking
bed sharing
hyperthermia and head covering
prematurity
320
How does DDH present
Usually diagnosed in infancy by screening tests. May be bilateral, when disease is unilateral there may be leg length inequality. As disease progresses child may limp and then early onset arthritis. More common in extended breech babies.
321
What are features of Ataxic CP
caused by damage to the cerebellum with typical cerebellar signs
poor coordination and balance
322
Do you need exclusion for measles
4 days after rash
323
What do you see in congenital CMV
Low birth weight
Purpuric skin lesions
Sensorineural deafness
Microcephaly
Visual impairment
Learning disability
Encephalitis/seizures
Pneumonitis
Hepatosplenomegaly
Anaemia
Jaundice
Cerebral palsy
324
What do you see in conegnital toxoplasmosis
Cerebral calcification
Chorioretinitis
Hydrocephalus
Anaemia
Hepatosplenomegaly
Cerebral palsy
325
Is there exclusion for scarlet fever
return 24 hours after starting antibiotics
326
When does TOF present
1-2 months but sometimes not picked up till 6 months
