Paeds Flashcards
(445 cards)
1
Q
List conditions associated with Down’s syndrome (2)
A
Hypothyroidism > hyperthyroidism
T1DM
2
Q
Threshold for hypoglycaemia
A
<2.6mmol/L
3
Q
Causes of persistent/severe hypoglycaemia in neonates
A
Preterm birth (<37wks), IUGR
Hypothermia, neonatal sepsis, inborn errors of metabolism, nesidioblastosis, Beckwith-Wiedemann syndrome
4
Q
Symptoms of neonatal hypoglycaemia (4)
A
May be asymptomatic
Hypoglycaemia (changes in neural sympathetic discharge): jitteriness, irritable, tachypnoea, pallor
Neuroglycopenic (shortage of glucose in the brain): poor feeding/sucking, weak cry, drowsy, hypotonia, seizures
Others: apnoea, hypothermia
5
Q
Management of neonatal hypoglycaemia
A
Asymptomatic: encourage normal breastfeeding, monitor glucose
Symptomatic (<2) or very low glucose (<1): admit to neonatal unit, IV infusion of 10% dextrose
6
Q
Features of benign rolandic epilepsy (7)
A
Partial seizures occurring at night
Hemifacial paraethesias
Secondary generalisation to tonic-clonic seizures
Oropharyngeal manifestation (strange noises), hypersalivation
FHx
Otherwise normal, good prognosis, usually stops by adolescence
7
Q
EEG manifestation of benign rolandic epilepsy
A
Centrotemporal spikes
8
Q
Empirical Mx for bacterial meningitis (5)
A
- Abx
<3m : IV amoxicillin/ ampicillin + IV cefotaxime
>3m : IV cefotaxime/ ceftriaxone - Steroids (AVOID corticosteroids in babies <3m)
Dexamethasone
if LP: purulent CSF, CSF WCC >1000/microlitre, raised CSF WCC with protein conc >1g/L, bacteria on gram stain - Fluids
Treat any shock e.g. with colloid - Cerebral monitoring
Mechanical ventilation if resp impairment - Public health notification and ABx prophylaxis of contacts
Ciprofloxacin»_space; rifampicin
9
Q
Symptoms of cystic fibrosis (7)
A
Recurrent chest infections
Malabsorption: steatorrhoea (due to pancreatic insufficiency, malabsorption of fats), failure to thrive (short stature, delayed puberty)
Meconium ileus
Liver disease, DM
Rectal prolapse
Nasal polyps
Male infertility, female subfertility
10
Q
List causes of constipation in children (6)
A
Idiopathic
SECONDARY TO ANXIETY
Dehydration, low fibre diet
Medication: opiates
Anal fissure
Over-enthusiastic potty training
Hypothyroidism, Hirschsprung’s disease, hypercalcaemia, learning disabilities
11
Q
What should be assessed before starting treatment for constipation?
A
Check for faecal impaction: Sx of severe constipation, overflow soiling, faecal mass palpable in the abdomen (DRE only by specialist)
12
Q
Treatment plan if faecal impaction is present
A
- Osmotic laxative, escalating dose
- Add stimulant if no disimpaction after 2 weeks
- Inform families that disimpaction therapy may lead to increase in soiling and abdominal pain
13
Q
Most common fractures associated with child abuse
A
Radial, humeral, femoral
14
Q
Paediatrics constipation maintenance therapy
A
MSO
Movicol Paediatric Plan (polyethylene glycol 3350 + electrolytes)
Senna (stimulant)
Osmotic (lactulose) if stools are hard despite the top two meds
Continue the maintenance therapy for several weeks after regular bowel habit is established
15
Q
Why should evidence of exomphalos in an antenatal scan indicate elective C-section?
A
To reduce risk of sac rupture, infection and atresia secondary to injury
16
Q
Difference between gastroschisis and exomphalos
A
In exomphalos (omphalocele), the abdominal contents protrude through anterior abdominal wall but it’s covered in an amniotic sac formed by amniotic membrane and peritoneum. C-section is indicated to reduce risk of sac rupture, staged closure can be undertaken as primary closure may be difficult due to high intra-abdominal pressure/lack of space.
In Gastroschisis the contents are not in a peritoneal covering. Vaginal delivery can be trialled, neonate should be taken to surgery after delivery within 4 hours.
17
Q
Define scarlet fever
A
Reaction to erythrogenic toxins produced by Group A haemolytic streptococci (strep pyogenes)
18
Q
Epidemiology of scarlet fever
A
Common in children aged 2-6yo with peak incidence at 4yo
19
Q
Route of transmission of scarlet fever
A
Respiratory route by inhaling/ingesting respiratory droplets or by direct contact with nose and throat discharges esp during coughing and sneezing
20
Q
S+Sx of scarlet fever
A
Incubation time of 2-4days
Fever lasting 24-48hrs
Malaise, headache, n&v
Sore throat
Strawberry tongue, rash
21
Q
Describe the rash present in scarlet fever
A
Fine punctuate erythema (pinhead), generally appearing first on the torso, sparing the palms and soles, more prominent in flexures. Rough, sad-paper texture.
Flushed appearance with circumoral pallor.
Desquamination occurs later in the course of illness, esp around fingers and toes.
22
Q
Ix for scarlet fever
A
Throat swab but start Abx treatment STAT
23
Q
Mx for scarlet fever
A
Oral penicillin V for 10 days or azithromycin if penicillin allergy.
Children can return to school after 24hrs of starting Abx.
Notifiable disease!
24
Q
Most common complication of scarlet fever
A
Otitis media!!
Others: rheumatic fever (around 20days post infection), acute glomerulonephritis
Rare: invasive complications e.g. bacteraemia, meningitis, necrotizing fasciitis are rare but present with acutely life-threatening illness.
25
4 signs of Noonan syndrome
Webbed neck, pectus excavatum, short stature, pulmonary stenosis
26
4 signs of Patau syndrome (trisomy 13)
Microcephalic, small eyes
Cleft lip/palate
Polydactyly
Scalp lesions
27
5 signs of Fragile X syndrome
Learning difficulties
Macrocephaly
Long face
Large ears
Macro-orchidism
28
What are febrile convulsions and what age range is it normally seen?
Seizures provoked by fever in otherwise normal children. Most common in 6months-5yo (3%).
29
Key features of febrile convulsions (3)
Occur early after viral infection as temp rises rapidly. Seizures usually brief, lasting 5mins. Most tonic clonic.
30
Types of febrile convulsions
Simple: <15mins, generalised seizure, complete resolves in 24hrs, no recurrence
Complex: 15-30mins, focal seizure, may have repeat seizures within 24hrs
Focal status epilepticus
31
Mx following a seizure
If first ever seizure/ complex, admit to paediatrics ASAP
32
Ongoing Mx for febrile convulsions / seizure
1. If seizure lasts >5mins, call ambulance
2. Regular antipyretics have not been shown to reduce chance of febrile convulsions
3. If recurrent febrile convulsions occur, BDZ rescue medication should be considered: rectal diazepam/buccal midazolam
33
What medication should you avoid in chickenpox?
NSAID e.g. ibuprofen due to increase in secondary bacterial infection of the lesions (necrotising fasciitis)
34
What is chickenpox and shingles?
Chickenpox is primary infection with varicella zoster virus. Shingles is reactivation of the dormant virus in the dorsal root ganglion.
35
How infective is chickenpox?
Via respiratory route. Can be caught from someone with shingles. Incubation period of 10-21 days. Infectivity occurs 4 days before onset of rash and 5days after rash appears.
36
S+Sx of chickenpox
Initial fever
Itchy rash, starting on head/trunk before spreading. Macular --> papular --> vesicular
37
Mx of chickenpox
Keep cool, trim nails
Calamine lotion
Exclusion from school until end of infectivity period
38
Rare complications of chickenpox
Pneumonia, encephalitis, disseminated haemorrhagic chickenpox
Arthritis, nephritis, pancreatitis
39
Action for child <3yo presenting with acute limp
Urgent specialist assessment
40
DDx for limping child
Transient synovitis: acute, viral infection, mild fever, M>F, 2-12yo
Septic arthritis/osteomyelitis: unwell, high fever
Juvenile idiopathic arthritis: painless limp
Trauma: Hx diagnostic
Development of dysplasia of hip: detected in neonates, 6x more common in girls
Perthes disease: 4-8yo, due to avascular necrosis of the femoral head
Slipped upper femoral epiphysis: 10-15yo, displacement of femoral head epiphysis postero-inferiorly
41
What is the purpose of traffic light system for fever in child?
Risk stratification for children <5yo.
Includes colour, activity, respiratory , circulation and hydration.
42
Red flag signs for feverish child
Pale, blue
No response, does not wake, weak high-pitched/ continuous cry, grunting, chest retraction, RR>60, REDUCED SKIN TURGOR, age<3months, non blanching rash, bulging fontanelle, neck stiffness, focal neurological signs, focal seizures
43
Define precocious puberty
Development of secondary characteristics before age 8 in female and age 9 in male
44
Define thelarche
First stage of breast development
45
Define adrenarche
First stage of pubic hair development
46
What is Osgood-Schlatter disease?
Inflammation of growth plate at the tibial tuberosity causing anterior knee pain in adolescence
47
Which demographic is mostly affected by slipped capital femoral epiphysis?
Obese, boys, 10-15yo
48
S+Sx of slipped capital femoral epiphysis
Hip, groin, medial thigh/knee pain
Loss of internal rotation of leg in flexion, bilateral slip in 20% cases
49
Ix for slipped capital femoral epiphysis
AP + lateral (typically frog leg) views are diagnostic
50
Mx of slipped capital femoral epiphysis
Internal fixation: a single cannulated screw placed in centre of epiphysis
51
Complications of slipped capital femoral epiphysis
Osteoarthritis, avascular necrosis of femoral head, chondrolysis, leg length discrepency
52
Key features of Down's syndrome
Upslanting palpebral fissures, epicantic folds, Brushfield spots in iris, protrudng tongue, small low-set ears, round/flat face
Flat occiput
Single palmar crease, sandal gap between big and first tow
Hypotonia
Congenital heart defects
Duodenal atresia
Hirschsprung's disease
53
List 4 features of Trisomy 18 (Edward's syndrome)
Micrognathia, low-set ears, rocker bottom feet, overlapping of fingers
54
Define nocturnal enuresis
Involuntary discharge of urine by day/night/both, in a child aged 5 years or older, in the absence of congenital/acquired defects of the nervous system/urinary tract
55
Difference between primary and secondary nocturnal enuresis
Primary: child has never achieved continence
Secondary: child has been dry for at least 6m before
56
List the Mx options for nocturnal enuresis
1. Look for possible underlying cause: constipation, DM, UTI if acute
2. General advice: reduce fluid intake before night, encourage bladder emptying regularly during the day and before sleep, starting a reward system for agreed behaviour (star charts) rather than dry nights e.g. using toilet to pass urine before sleep
3. Enuresis alarm: have sensor pads that sense wetness (high success rate)
4. Desmopressin for short-term control/ enuresis alarm as been ineffective/not acceptable to family
57
What organism causes necrotising fasciitis in a patient with chickenpox?
Group A streptococcal soft tissue infections affecting soft tissues
58
List causes of chronic diarrhoea (4)
Cow's milk intolerance
Toddler diarrhoea
Coeliac disease
Post-gastroenteritis lactose intolerance
59
When is ultrasound screening of the hip conducted?
6 weeks of age for newborns with specific risk factors
60
What is developmental dysplasia of the hip (DDH)?
Congenital dislocation of the hip, affecting 1-3% of newborns
61
List 7 risk factors for DDH
F>M (6x)
Breech presentation, oligohydraminos
Positive FHx
Firstborn children
Birthweight >5kg
Calcaneovalgus foot deformity
62
Which side is more commonly affected in DDH?
Left. Around 20% of cases are bilateral
63
List three RFs that indicate a routine US examination for DDH
First degree FHx of hip problems in early life
Breech presentation at or after 36wks gestation irrespective of birth or mode of delivery
Multiple pregnancy
64
Clinical examination of DDH
Barlow test: attempt dislocation of articulated femoral head
Ortolani test: attempt relocation of dislocated femoral head
Other factors: symmetry of leg length, level of knees when hips and knees are bilaterally flexed, restricted abduction of hip in flexion
65
Imaging used for DDH
??clinically suspected, confirm diagnosis --> USS
If >4.5m, FIRST LINE IS XRAY
66
Mx of DDH
Most unstable hips stabilise by 3-6wks of age
Pavlik harness (dynamic flexion-abduction orthosis) in children younger than 4-5m
Older children may require surgery
67
Define Perthes' Disease
Degenerative condition affecting hip joints of children, due to avascular necrosis of femoral head, specifically the femoral epiphysis (impaired blood supply causes infarction)
68
S+Sx of Perthes' disease
Hip pain: progressively over a few weeks
Limp
Stiffness and reduced range of hip movement
69
X-ray features of Perthes' disease
Early changes include widening of joint space, later changes include decreased femoral head size/flattening
70
Ix for Perthes' disease
Plain X-ray
Technetium bone scan or MRI if normal X-ray and Sx persist
71
Complications of Perthes' disease
Osteoarthritis
Premature fusion of growth plates
72
Define reflex anoxic seizures
Syncopal episode (presyncope) that occurs in response to pain or emotional stimuli. Thought to be caused by neurally-mediated transient asystole in children with very sensitive vagal cardiac reflexes. Most common in 6m-3yo.
73
List 4 S+Sx of reflex anoxic seizures
Pale, falls to floor
Stiffness
Rapid recovery (key difference to epilepsy)
74
Tx and prognosis of reflex anoxic seizures
No specific treatment, prognosis is excellent
75
Name a common side effect of ventouse delivery
Caput succedaneum
76
What is Caput succedaneum?
Oedema of the scalp at the presenting part of the head, typically at the vertex. May be due to mechanical trauma of the initial portion of scalp pushing through cervix in a prolonged delivery or secondary to use of ventouse (vacuum) delivery.
77
S+Sx of necrotising fasciitis
Feeding intolerance
Abdominal distension, bloody stools
Abdominal discolouration, perforation, peritonitis
78
Signs on AXRs that indicate necrotising enterocolitis (7)
Asymmetrical dilated bowel loops
Bowel wall oedema
Pneumatosis intestinalis (intramural gas)
Portal venous gas
Pneumoperitoneum from perforation
Air both inside and outside of bowel wall (Rigler sign)
Air outlining the falciform ligament (football sign)
79
Pathogen responsible for bronchiolitis (in 75-80% of cases)
RSV respiratory syncytial virus
Other causes: mycoplasma, adenoviruses
80
Epidemiology of bronchiolitis
Most common cause of serious low respiratory tract infection in <1yo, peach incidence at 3-6m olds.
Higher incidence during the winter.
81
S+Sx of bronchiolitis (5)
Coryzal Sx including mild fever precede:
Dry cough
Increase breathlessness
Wheezing, fine inspiratory crackles
Feeding difficulties associated with increasing dyspnoea (often the reason for admission)
82
What should warrant an urgent referral (999) for bronchiolitis (5)?
Apnoea (observed/reported)
Child looks seriously unwell to a healthcare professional
Severe respiratory distress: grunting, marked chest recession, RR<70breaths/min
Central cyanosis
Persistent O2 sats of <92% when breathing air
83
When should you consider referring a suspected bronchiolitis child to hospital (4)?
RR >60
Difficulty breastfeeding or inadequate oral fluid intake
Clinical dehydration
84
Ix for bronchiolitis
Immunofluorescence of nasopharyngeal secretions may show RSV
85
Mx of bronchiolitis
Largely supportive
Humidified O2 given via head box and is typically recommended if O2 sats are persistently <92%
Nasogastric feeding may be needed if children cannot take enough fluid/feed by mouth
Suction is sometimes used for excessive upper airway secretions
86
What is Hirschsprung's disease?
Aganglionic segment of bowel due to developmental failure of parasympathetic Auerback and Meissner plexuses
87
How common is Hirschprung's disease?
1 in 5,000 births RARE but an important Dx for constipation in childhood
More common in males
88
S+Sx of Hirschsprung's disease
Neonatal period e.g. failure or delay to pass meconium
Other children: constipation, abdominal distension
89
Ix for Hirschsprung's disease
AXR
Rectal biopsy : GOLD
90
Mx of Hirschsprung disease
Initially: rectal washouts/bowel irrigation
Definitive Mx: surgery to affected segment of colon
91
Describe the rash in ezcema
Itchy, erythematous rash
Repeated scratching may exacerbate the affected areas
92
Describe the distribution of ezcema in infants, younger children, older children
Infants : face and trunk
Younger children : eczema on extensor surfaces
Older children : flexor surfaces affected and creases of the face and neck
93
Mx of eczema
Avoid irritants
Simple emollients: large quantities prescribed (250g/week), apply emollient 30mins before applying the steroid, creams soak into skin faster than ointments, emollients can become contaminated with bacteria (fingers should not be inserted into pots, many have pump dispensers)
Topical steroids
Wet wrapping: large amounts of emollient (sometimes topical steroids) applied under wet bandages
In severe cases, oral ciclosporin may be used
94
Characteristics of infantile colic (4)
Typical in infants <3m
Excessive crying
Pulling up of legs
Often worse in evenings
95
What is NOT recommended for infantile colic?
Simeticone/lactase drops
96
Epidemiology of ALL
Peak incidence 2-5yo
M>F
Most common malignancy affecting children
Not a strong family correlation (although Down's increase likelihood)
97
Key features of ALL
Anaemia : pallor, lethargy
Neutropenia : frequent/severe infections
Thrombocytopenia : easy bruising, petechiae
Bone pain (secondary to bone marrow infiltration), splenomegaly, hepatomegaly, fever
98
Poor prognostic factors of ALL
Age <2yo or >10
WBC >20X10^9/l at Dx
T/B cell surface markers
Non-Caucasian
Male sex
99
School exclusion rules for chicken pox
Until all the lesions have crusted over, which occur 5 days after onset of rash
100
Areas affected by seborrhoeic dermatitis
Scalp, nappy area, face, limb flexures
101
Early sign of seborrhoeic dermatitis
Early sign that develops in the first few weeks of life, erythematous rash with coarse yellow scales
102
Mx of seborrhoeic dermatitis
Reassure that it doesn't affect baby and usually resolves within few weeks (tends to resolve around 8m of age)
Massage topical emollient onto the scalp to loosen scales, brush gently with soft brush and wash off with shampoo
If severe/persistent a topical imidazole cream may be tried
103
Triad for shaken baby syndrome
Retinal haemorrhages, subdural haematoma, encephalopathy
104
What causes shaken baby syndrome?
Caused by intentional shaking of a child 0-5yo
105
What is Meckel's diverticulum?
Congenital diverticulum of the small intestine. It is a remnant of the omphalomesenteric duct containing ectopic ileal, gastric or pancreatic mucosa.
106
Rule of 2s for Meckel's diverticulum
Occurs in 2% of population
2 feet from the ileocaecal valve
2 inches long
107
S+Sx of Meckel's diverticulum (3)
Usually asymptomatic
Abdominal pain (mimik appendicitis)
Rectal bleeding (MOST COMMON CAUSE OF MASSIVE PAINLESS GI BLEEDING requiring transfusion in children between ages of 1-2yo)
Intestinal obstruction secondary to an omphalomesenteric band, volvulus, intussusception
108
Ix for Meckel's diverticulum
If child is haemodynamically stable with less severe of intermittent bleeding then a Meckel's scan should be considered
Mesenteric arteriography also may be used in more severe cases e.g. transfusion is required
109
Mx of Meckel's diverticulum
Removal if narrow neck or symptomatic
Either wedge excision or formal small bowel resection and anastomosis
110
Mx of Perthes' disease
Keep femoral head within the acetabulum: cast, braces
If less than 6yo: observation
If older: surgical Mx with moderate results
Operate on severe deformities
111
Prognosis of Perthes' disease
Most cases resolve with conservative Mx, early Dx improves outcomes
112
Define neonatal sepsis
Serious bacterial/viral infection in the blood affecting babies within the first 28days of life
113
Difference between early onset and late onset sepsis
Early onset : within 72hrs of birth
Late onset : between 7-28days of life
114
Cause of EOS
GBS infection (75%) usually due to transmission of pathogens from the mother to the neonate during delivery
115
Cause of LOS
Staphylococcus epidermis
Pseudomonas aeruginosa
Klebsiella and Enterobacter
Due to transmission of pathogens from environment post delivery, from parents/healthcare workers
116
RFs for neonatal sepsis
Mother with previous GBS infection, intrapartum temp >=38deg, membrane rupture >=18hrs, current infection during preg
Premature birth (<37wks)
Low birth weight (<2.5kg)
Evidence of maternal chorioamnionitis
117
Ix for neonatal sepsis
Blood culture to obtain Dx
FBC to exclude healthy neonates
CRP guide Mx and patient progress during treatment
Blood gas : metabolic acidosis, BE -10>=
Urine microscopy, culture, sensitivity (?UTI? for LOS)
LP ?meningitis, or part of septic screen
118
Mx of neonatal sepsis
Main aim: improve outcomes by early identification and Tx
Use IV benzylpenicillin + gentamicin as first-line regimen for suspected/confirmed
Measure CRP 18-24hrs after Abx to monitor progress and guide duration of therapy
Cease Abx in neonates CRP <10mg/L and negative blood culture at presentation and at 48hrs
Maintain O2, normal fluid and electrolyte status
Monitor body weight for assessment of fluid status
Prevent and manage hypoglycaemia and metabolic acidosis
119
What is Kawasaki disease?
Type of vasculitis predominantly seen in children
120
Name a serious complication of Kawasaki disease
Coronary artery aneurysms
121
S+Sx of Kawasaki disease
High grade fever (lasting >5days, RESISTANT TO ANTIPYRETICS)
Conjunctival injection
Bright red, cracked lips
Strawberry tongue
Cervical lymphadenopathy
Red palms of the hands and soles of feet which later peel
122
Dx test of Kawasaki disease
No specific diagnostic test, it is clinically diagnosed!
123
Mx of Kawasaki disease
High dose aspirin
IV immunoglobulin
Echocardiogram used as an initial screening test for coronary artery aneurysms
124
S+Sx 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 and confluent, desquamination that typically spares the palms and soles may occur after a week
Diarrhoea around 10% of patients
125
Ix for measles
IgM Abs detected within a few days of rash onset
126
Mx of measles
Supportive
Admission if immunosuppressed/ pregnant
Notifiable disease --> public health
127
Main complication of measles (3)
Otitis media : most common
Pneumonia : most common cause of death
Encephalitis : typically occurs 1-2wks following onset of illness
128
Mx of a child that came into contact with measles
MMR vaccine induced Abs develops more rapidly than that following infection
Give within 72hrs
129
What is the commonest cause of vomiting in infancy?
GORD
130
RFs of GORD
Preterm delivery, neurological disorders
131
Features of GORD in children (3)
Typically develops before 8wks
Vomiting/regurg (milky vomit after feeds, may occur after being laid flat)
Excessive crying, especially while feeding
132
Dx of GORD
Made clinically
133
Mx of GORD
1. Advice regarding position during feeds : 30deg head-up
2. Infants should sleep on their backs to reduce risk of cot death
3. Make sure infant is not being overfed : trial smaller feeds/ more frequent
4. Trial of thickened formula : rice starch, cornstarch
5. Trial alginate therapy e.g. Gaviscon NOT with thickening agents
6. PPI if 1 applies : unexplained feeding difficulties (refuse feeds, gag, choke), distressed behaviour, faltering growth
7. Prokinetic agents e.g. metoclopramide should be used with specialist advice
134
Complications of GORD in infants (5)
Failure to thrive
Distress
Aspiration
Frequent otitis media
In older children, dental erosion may occur
135
What to do with severe complications of GORD?
If medical Tx is ineffective, consider fundoplication
136
Define intussusception
Invagination of one portion of the bowel into the lumen of the adjacent bowel, most commonly around the ileocaecal region
137
Epidemiology of intussusception
6-18months old
M>F (x2)
138
S+Sx of intussusception (6)
Intermittent, severe, crampy, progressive abdo pain
Inconsolable crying
During paroxysm the infant will characteristically draw their knees up and turn pale
Vomiting
Blood stained stool - red current jelly (LATE sign)
Sausage shaped mass in the RUQ
139
Ix for intussusception
USS may show target like mass
140
Mx of intussusception
Tx with reduction by air insufflation under radiological control, widely used first-line compared to traditional barium enema
If this fails/peritonitis --> surgery
141
Inheritance pattern of achondroplasia
AD
142
S+Sx of achondroplasia (5)
Short limbs (rhizomelia), shortened fingers (brachydactyly)
Large head with frontal bossing and narrow foramen magnum
Midface hypoplasia with flattened nasal bridge
Trident hands
Lumbar lordosis
143
RFs for achondroplasia
70% due to sporadic mutation
Advanced paternal age at the time of conception
144
Tx for achondroplasia
No specific therapy, but some benefit from limb lengthening procedures - application of llizarov frames and targeted bone fractures
145
What should be screened for Down's syndrome infant taking part in excercise?
Atlanto-axial instability due to high risk of neck dislocation
(a complication of DS is atlanto-axial instability)
146
What is congenital diaphragmatic hernia?
Occurs 1 in 2,000 newborns, characterised by herniation of abdominal viscera into chest cavity due to incomplete formation of diaphragm
147
Most common type of CDH
Left sided posterolateral Bochdalek hernia for around 85% of cases
148
Prognosis of CDH
50% of newborns survive despite modern medical intervention
149
S+Sx of CDH
Pulmonary hypoplasia and HTN causes respiratory distress shortly after birth
150
Define croup
Form of upper respiratory tract infection in infants and toddlers, characterised by stridor caused by combination of laryngeal oedema and secretions.
151
Epidemiology of croup
6m-3yo, more common in autumn
152
S+Sx of croup (4)
Stridor
Barking cough
Fever
Coryzal Sx
153
When should a child with croup be admitted?
Moderate-severe
OR
* if <6mo / known upper airway abnormality / uncertainty about Dx --> admit!!
154
X-ray sign of croup
Steeple sign : PA view showing subglottic narrowing
155
Mx of croup
Regardless of severity
Single dose of oral dexamethasone 0.15mg/kg STAT (prednisolone alternatively)
High flow O2, nebulised adrenaline if emergency!
156
Test done as part of Newborn Hearing Screening Programme
Otoacoustic emission test - done prior to being discharged from hospital.
Soft ear piece placed in baby's ear and quiet clicking noises are played through it. Earpiece picks up the response from the inner ear and computer analyses it.
157
Test done if otoacoustic test is abnormal
Auditory Brainstem Response (3 sensors placed on head and neck; soft headphones used to play quiet clicking sounds; sensors detect how baby's brain and hearing nerves respond to sound and computer analyses results)
158
List factors that point towards child abuse (4)
Story inconsistent with injuries
Repeated attendances at A&E departments
Delayed presentation
Child with a frightened, withdrawn appearance - 'frozen watchfulness'
159
Possible physical presentations of child abuse (6)
Bruising
Fractures: multiple at different stages of healing, posterior rib fractures
Burns and scalds
Failure to thrive
STIs
160
Define microcephaly
Occipital-frontal circumference <2nd centile
161
List causes of micocephaly (7)
Normal variation
Familial e.g. parents have small head
Congenital infarction
Perinatal brain injury e.g. hypoxic ischaemic encephalopathy
Fetal alcohol syndrome
Patau syndrome
Craniosynostosis
162
Mx for child presenting with ADHD
1. Watch and wait period of 10wks
2. If persists, refer to paediatrician/CAMHS
3. Drug therapy seen as last resort for >5yo
Methylphenidate FIRST LINE 6wk trial
If inadequate, switch to lisdexamfetamine but if this causes SEs give dexamfetamine
163
SEs of methylphenidate (ADHD first line) in children
Abdo pain, nausea, dyspepsia
Note: weight and height should be monitored every 6m
164
How harmful are ADHD drugs and what should be monitored?
These are cardiotoxic drugs, so perform baseline ECG before starting and refer to cardiologist if there is significant PMHx, FHx
165
Define vesicoureteric reflux (VUR)
Abnormal backflow of urine from bladder into ureter and kidney
166
Epid of VUR
Common abnormality of urinary tract in children and predisposes to UTI (30% of UTI have VUR) and 35% develop renal scarring so it is important to Ix for VUR following UTI.
167
List presentations of VUR (3)
Antenatal period : hydronephrosis of USS
Recurrent childhood UTI
Reflux nephropathy : term used to describe chronic pyelonephritis secondary to VUR, commonest cause of chronic pyelonephritis, renal scar may produce increased quantities of renin causing HTN
168
Ix for VUR (2)
Micturating cystourethrogram
DMSA scan may be used to look for renal scarring
169
List 5 grades of VUR
1. Reflux into ureter only, no dilatation
2. Reflux into renal pelvis on micturition, no dilatation
3. Mild/moderate dilatation of ureter, renal pelvis and calyces
4. Dilation of renal pelvis and calyces with moderate ureteral tortuosity
5. Gross dilatation of ureter, pelvis, calyces with ureteral tortuosity
170
Epid of growing pains
M=F
3-12yo
171
S+Sx of growing pains (7)
Never present at the start of day
No limp, no limitation of physical activity
Systemically well, normal examination
Motor milestones normal
Sx intermittent and worse after a day of vigorous activity
172
List causes of jaundice in the first 24hrs (4)
ALWAYS PATHOLOGICAL
Rhesus haemolytic disease
ABO haemolytic disease
Hereditary spherocytosis
Glucose-6-phosphodeydrogenase
Measure serum bilirubin urgently!!
173
Causes of jaundice in neonates from 2-14days
Common (up to 40%)
Usually physiological (more RBCs, less developed liver function)
More common in breastfed babies (?? high conc of beta-glucuronidase --> increase in intestinal absorption of unconjugated bili)
174
List Ix for jaundice after 14days (prolonged, or 21days in premature)
Conjugated and unconjugated bilirubin (? biliary atresia requires urgent surgical intervention)
Direct antiglobulin test
TFTs
FBC and blood film
Urine for MC&S and reducing sugars
U&Es and LFTs
175
List causes of prolonged jaundice (7)
Biliary atresia
Hypothyroidism
Galactosaemia
UTI
Breast milk jaundice
Prematurity (immature liver function, increased risk of kernicterus)
Congenital infections e.g. CMV, toxoplasmosis
176
Classical S+Sx of slipped upper femoral epiphysis (SUFE)
Obese, young boys (10-15yo) (displacement of femoral head epiphysis posteriorinferiorly)
Hip, groin, medial thigh +/- knee pain. Loss of internal rotation of leg, whilst flexion may be seen)
177
Presentation of UTI in childhood (infants, younger children, older children)
Infants: poor feeding, vomiting, irritability
Younger children: abdominal pain, fever, dysuria
Older children: dysuria, frequency, haematuria
Fts of upper UTI: temp ?38deg, loin pain/tenderness
178
List 3 scenarios of when a child's urine sample should be obtained
Sx/S of UTI
Unexplained fever of 38deg + (test urine after24hrs the latest)
With an alternate site of infection but remains unwell (consider urine test after 24hrs at the latest)
179
3 different ways of collecting urine from children
Preferred method: clean catch
Urine collection pads
Don't use cotton balls, gauze, sanitary towels
Invasive methods e.g. suprapubic aspiration should only be used if non-invasive methods are not possible
180
Mx of UTI
<3m should be referred immediately to paediatrician
>3m + upper UTI consider admission to hospital / if not admitted, five oral e.g. cephalosporin/co-amoxiclav for 7-10days
>3m + lower UTI Tx with Abx for 3days according to local guidelines e.g. trimethoprim, nitrofurantoin, cephalosporin, amox)
Bring child back if unwell for 24-48hrs
Abx prophylaxis considered with recurrent UTIs
181
Define meconium aspiration syndrome
Resp distress in newborn as a result of meconium in trachea
182
RFs of meconium aspiration syndrome
Post-term deliveries (44% in babies born after 42wks)
Maternal HTN, pre-eclampsia, chorioamnionitis, smoking/substance abuse
183
Causes of hypotonia (7)
Down's syndrome
Prader Willi syndrome
Hypothyroidism
Cerebral palsy (hypotonia may precede development of spasticity)
Spinal muscular atrophy
GBS
MG
184
Name the most common congenital infection in the UK
CMV, rubella, toxoplasmosis
185
List S+Sx of congenital CMV infection
Low birth weight
Petechial rash
Sensorineural deafness
Seizure
Jaundice
Microcephaly
Learning disability
186
Two prognostic factors of CDH
Liver position : if liver has herniated to the chest, disease is more severe and lower chance of survival
lung-to-head ratio : >1.0 reflects better outcome
187
When does a baby start to smile?
6wks
188
Organisms causing meningitis in neontal-3m
Group B strep (acquired from mother at birth), E coli, Listeria monocytogenes
189
Organisms causing meningitis in 1m to 6m
Neisseria meningitidis
Streptococcus pneumoniae
Haemophilus influenzae
190
Organisms causing meningitis in >6yrs
Neisseria meningitidis
Streptococcus pneumoniae
191
When is Meningitis B vacc given?
2m, 4m, 12-13m
192
Cause of hand, foot and mouth disease
Coxsackie A16 and enterovirus 71 (contagious, typically outbreaks in nursery)
193
S+Sx of Coxsackie A16 disease (3)
Mild systemic upset : sore throat, fever
Oral ulcers
Followed later by vesicles on palms and soles of feet
194
Mx of Coxsackie infection (3)
Sx Tx ONLY : general advice about hydration and analgesia
Reassurance there is no link to cattle
No exclusion from school : stay home until they feel better, contact HPA if there is a large outbreak
195
Define therapeutic cooling
Deliberate lowering of patient's body temperature to prevent brain damage
196
Prognosis of HIE
10-60% die during neonatal period, 25% suffer from long term neurological impairment including epilepsy, mental retardation/ cerebral palsy.
197
When should therapeutic cooling be started?
Within 6hrs of birth and continued for 72hrs ; then cooling rewarming should be carried out slowly 0.5deg over a period of 6-12hrs
198
Define phimosis
Non-retractable foreskin +/- ballooning during micturition
199
Mx of phimosis
<2 - expectant approach
Avoid forcible retraction as it can cause scar formation
Ensure personal hygiene
Consider Tx if >2, recurrent UTI, recurrent balanoposthitis
200
When to refer for developmental problems (3)
No smile at 10wks
Cannot sit unsupported at 12m
Cannot walk at 18m
201
Define Roseola infantum
Common disease of infancy, caused by HHV6. Incubation period of 5-15days and typically affects children aged 6m-2yo
202
S+Sx of roseola infantum (5)
High fever: last few days followed LATER BY A
Maculopapular rash
Nagayama spots : papular enanthem on uvula and soft palate
Febrile convulsions occur around 10-15%
Diarrhoea and cough commonly seen
203
List 3 consequences of HHV6 infection
Roseola infantum
Aseptic meningitis
Hepatitis
204
School exclusion for roseola infantum?
No
205
What is Duchenne muscular dystrophy?
X-linked recessive disorder due to a problem in the dystrophin gene required for normal muscular function
206
S+Sx of Duchenne muscular dystrophy (4)
Progressive proximal muscle weakness from 5yrs
Calf pseudohypertrophy
Gower's sign: child uses arms to stand up from a squatted position
30% of patients have intellectual impairment
207
Ix for Duchenne muscular dystrophy
Raised Cr kinase
DEFINITIVE DX: genetic testing
208
Mx of Duchenne muscular dystrophy
Largely supportive as there is currently no effective Tx
209
Prognosis of Duchenne muscle dystrophy
Most children cannot walk by age 12
Patients typically survive to 25-30yo
Associated with dilated cardiomyopathy
210
RFs of RDS (5)
Premature (<28wks)
C-section
Male
Second born of premature twins
211
S+Sx of RDS
Tachypnoea, intercostal recession, expiratory grunting and cyanosis
212
Ix of RDS
Ground-glass appearance with indistinct heart border
213
Mx of RDS
Prevention during pregnancy: maternal corticosteroids to induce fetal lung maturation
O2
Assisted ventilation
Exogenous surfactant given via endotracheal tube
214
Mx of hypospadias
1. Refer to specialist services
2. Corrective surgery conducted when around 12m of age
3. Essential child is not circumcised prior to surgery
4. In boys with very distal disease, no Tx may be needed
215
List 4 key features of hypospadias
Ventral urethral meatus
Hooded prepuce
Chordee (ventral curvature of penis)
Urethral meatus may open more proximally in more severe variants
216
Define (periarticular) juvenile idiopathic arthritis
Arthritis occurring in someone who is <16yo that lasts >6wks
Periarticular JIA = <=4 joints affected (60%)
217
S+Sx of periarticular JIA (3)
Joint pain and swelling: usually medium sized joint e.g. knees, ankles, elbows
Limp
ANA may be positive - associated with anterior uveitis
218
Define biliary atresia
Obstruction/discontinuity within extrahepatic biliary system which results in cholestasis in the first weeks of life
219
S+Sx of biliary atresia
Jaundice extending from the physiological 2wks
Dark urine, pale stools
Appetite and growth disturbance
Hepatomegaly, splenomegaly, abnormal growth, cardiac murmurs
220
Ix of biliary atresia (6)
Serum bilirubin (conjugated>>unconjugated)
LFTs (raised but cannot differentiate biliary atresia and other causes of neonatal jaundice)
Serum alpha-1-antitrypsin: neonatal cholestasis
Sweat chloride test: cystic fibrosis
US of biliary tree and liver: ?distension
Percutaneous liver biopsy with intraoperative cholangioscopy
221
Mx of biliary atresia
ONLY DEFINITIVE - Kasai portoenterostomy:
Abx coverage and bile enchancers after pregnancy
222
Complications of biliary atresia (3)
Unsuccessful anastomosis formation, progressive liver disease, cirrhosis with eventual hepatocellular carcinoma
223
Mx of threadworms
Anthelmintic with hygiene measures for all members of household (mebendazole FIRST LINE for >6mo SINGLE DOSE unless infestation persists)
224
Most common place affected by cephalohaematoma
Parietal
225
Caput succedaneum vs cephalohaematoma
Caput succadaneum
present at birth, forms over vertex and CROSS SUTURE LINES, resolve within a few days
Cephalohaematoma
Several hrs after birth, parietal region, DOESNT CROSS SUTURE LINES, may take months to resolve
Similarities
Swelling on the head, prolonged difficult deliveries, Mx conservative
226
Paediatric GI disorders
Pyloric stenosis: projectile vomiting
Acute appendicitis
Mesenteric adenitis: central abdo pain, URTI
Intussusception: telescoping bowel, colicky pain, d&v, red jelly stool
Intestinal malrotation, feature in exomphalos, CDH
Hirschprung's disease: delayed passage of meconium, abdo distension
Biliary atresia: jaundice >14days, increased conjug bili
Oesophageal atresia
Necrotising enterocolitis: prematurity RF, abdo distension, bloody stool
227
Vaccination at 12-13yo
HPV
228
Define Epstein's pearl
Congenital cyst found in mouth, common on hard palate, commonly mistaken for tooth
229
Tx of Epstein's pearl
No Tx required as they tend to spontaneously resolve over course of a few weeks
230
S+Sx of PDA (5)
Left subclavicular thrill
Continuous machinery murmur
Large volume, bounding, collapsing pulse
Wide pulse pressure
Apex beat
231
Mx of PDA
Indomethacin/ibuprofen inhibits PG synthesis given to neonate
note: if associated with another congenital heart defect amenable to surgery then prostaglandin E1 is useful to keep the duct open until after surgical repair
232
S+Sx of bronchiolitis
Poor feeding, cough, fever
Crackles, wheezing, increased resp effort
233
What condition do the majority of meconium ileus patients have?
Cystic fibrosis
234
Epidemiology of Tetralogy of Fallot
Most common cause of cyanotic congenital heart disease. Presents 1-2m, not picked up until baby is 6m old.
235
4 features of TOF
Ventricular septal defect (VSD)
Right ventricular hypertrophy
Right ventricular outflow obstruction, pulmonary stenosis
Overriding aorta
236
What determines degree of cyanosis and clinical severity of TOF
Severity of right ventricular outflow obstruction
237
Other S+Sx of TOF
Cyanosis: hypercyanotic tet spells (tachypnoea, severe cyanosis), right-left shunt, right-sided aortic arch, CXR showing boot-shaped heart, ECG showing right ventricular hypertrophy
238
Mx of TOF
Surgical repair undertaken in two parts
Cyanotic episodes helped by BB to reduce infundibular spasm
239
Features of Pierre-Robin syndrome (3)
Micrognathia
Posterior displacement of the tongue (may result in upper airway obstruction)
Cleft palate
240
Name the hearing test done on children at >3yo
Pure tone audiometry (school entry in UK) - headphones on and raise hand when they hear a bleep
241
Mx for bronchiolitis
Humidified O2 via headbox, recommended if O2 sats persistently <92%
Nasogastric feed if children cannot take enough fluid/feed by mouth
Suction is sometimes used for excessive upper airway secretions
242
In paediatric BLS, where should you check the pulse (in an infant and child)?
Brachial, femoral in an infant
Femoral in a child
243
What is infantile spasms?
Type of childhood epilepsy presents in first 4-8m of life, more common in female infants.
244
S+Sx of infantile spasms (3)
Characteristic 'salaam' attacks: flexion of head, trunk, arms followed by extension of arms
Only lasts 1-2secs but may be repeated up to 50 times
Progressive mental handicap
245
Ix for infantile spasms
EEG: shows hypsarrythmia in 2/3rds infants
CT shows diffuse/localised brain disease in 70%
246
Mx of infantile spasms
Poor prognosis
Vigabatrin FIRST-LINE
ACTH also used
247
What condition is pneumatosis intestinalis a hallmark feature of?
It is presence of intramural gas
Necrotising enterocolitis (inflammation and necrosis of the bowel wall!)
248
Paediatrics BLS outline
Unresponsive?
Call for help
Open airways. look, listen, feel for breathing.
Give 5 rescue breaths
Check for signs of circulation
15 chest compressions:2 rescue breaths (100/120CP per min)
249
How should chest compressions be administered in infants and children?
In infants: use two-thumb encircling technique
In children (>1yo): compress the lower half of sternum with one hand
250
Describe the difference in clinical severity of alpha-thalassaemia
There are two separate alpha globulin genes in each chromosome.
If 1/2 alleles are affected: hypochromic, microcytic normal Hb level
If 3 alpha globulin alleles affected: hypochromic, microcytic anaemia with splenomegaly (HbH disease)
If 4 alpha globulin alleles are affected: death in utero (hydrops fetalis, Bart's hydrops)
251
Guideline on asthma treatment in 5-16yo
1. Newly diagnosed: SABA
2. Not controlled/newly Dx asthma with Sx >=3/wk/nighttime waking: SABA + low dose ICS (beclomethasone)
3. SABA + low dose ICS + LTRA
4. SABA + low dose ICS + LABA
5. SABA + switch ICS/LABA with MART
6. SABA + switch moderate dose ICS with MART
7. SABA +
increase ICS to paediatric high dose
trial additional drug (theophylline)
seek advice from HCP with expertise is asthma
252
When is the rotavirus immunisation given?
2 doses, first at 2m and then second at 3m
253
Two types of pre-school wheeze
Episodic viral wheeze: only when there is viral URTI and Sx free between episodes
Multiple trigger wheeze e.g. exercise, allergens and cirgarette smoke
254
Advice given to parents who has a child with pre-school wheeze
STOP SMOKING
255
Mx of episodic viral wheeze(3)
1. Tx is symptomatic only
2. FIRST LINE - SABA (Salbutamol)/ anticholinergic via spacer
3. Intermittent LTRA, intermittent inhaled CS, both
256
Mx of multiple trigger wheeze
Trial of either inhaled CS or LTRA (montelukast) typically for 4-8wks
257
List 5 major RFs of SIDS
Prone sleeping
Parental smoking
Bed sharing
Hyperthermia, head covering
Prematurity
Others: male, multiple birth, social classes IV+V, maternal drug use, incidence increase in winter
258
List three protective factors for SIDS
Breastfeeding
Room sharing (NOT BED SHARING)
Use of dummies (pacifiers)
259
Mx of Hirschprungs disease
Initially, rectal washouts, bowel irrigation. Then surgical Mx is DEFINITIVE, done to the affected segment of the bowel.
260
S+Sx of fetal alcohol syndrome
Acutely, may show alcohol withdrawal Sx (hypotonia, irritable, tremors)
Features: small palpebral fissures, thin vermillion border, smooth filtrum, learning difficulties, microcephaly
261
Most common cause of pneumonia in children
S pneumoniae
262
Mx of pneumonia
FIRST LINE is amoxicillin, but if mycoplasma/chlamydia suspected --> erythromycin or add it if no improvement with amox
If pneumonia associated with influenza is co-amoxiclav
263
Ix for bacterial meningitis
LP (avoid if any sign of increased ICP)
264
Signs of increased ICP (hence CI to LP)
Focal neurological signs
Papilloedema
Significant budging of fontanelle
DIC
Signs of cerebral herniation
265
S+Sx of Fragile X syndrome
Learning difficulties
Large low set ears, long thin face, high arched palate
Macroorchisim
Hypotonia
Autism more common
Mitral valve prolapse
266
Dx of Fragile X syndrome
Made antenatally by chorionic villus sampling/ aminocentesis
Analysis of no. of CGG repeats using restriction endonuclease digestion and Southern blot analysis
267
CF should minimise contact with ... to prevent cross infection
Burkholderia cepacia complex and pseudomonas aeruginosa
268
Key features of Kawasaki disease
High grade fever resistant to anti-pyretics (>5days fever)
Strawberry tongue, cervical lymphadenopathy
Red palms of hand and soles of feet that later peel
Bright red, cracked lips
269
School exclusion rules for D&V
Until Sx have settled for 48hrs
270
School exclusion rules for whooping cough
2 days after commencing Abx (or 21days from onset of Sx if no Abx)
271
Define cerebral palsy
Non-progressive lesion present in motor pathways in developing brain (most common cause of major motor impairment)
272
List 4 features of cerebral palsy
Abnormal tone early infancy
Delayed motor milestones
Abnormal gait
Feeding difficulties
Learning difficulties, epilepsy, squints, hearing impairments
273
Classification of different types of cerbral palsy (4)
Spastic
Dyskinetic
Ataxic
Mixed
274
Mx of cerebral palsy
MDT required as this is a CHRONIC condition
Tx for spasticity: oral diazepam, oral and intrathecal baclofen, botulinum toxin type A, orthopaedic surgery and selective dorsal rhizotomy
Anticonvulsants, analgesia as required
275
What is in 6 in 1 vaccine?
Diptheria, tetanus, whooping cough, polio, Hib, hepB
276
What vaccines are given at 2m?
6-in-1 vaccine, one dose of Men B, one dose of Rotavirus vaccine
277
Mx of asthma in <5yo
1. SABA
2. SABA + 8wk trial of moderate dose inhaled CS
3. SABA + paediatric low dose CS + LTRA
4. Stop LTRA and refer to an paediatric asthma specialist
278
What is MART?
Form of combined ICS and fast-acting LABA treatment (formoterol)
279
What constitutes a low, moderate, high dose ICS?
LOW : <=200micrograms budesonide
MODERATE : 200micrograms-400micrograms budesonide
HIGH : >400micrograms budesonide
280
Cause of acute epiglottitis
Haemophilus influenzae type B
281
S+Sx of acute epiglottitis
Rapid onset
High temp, generally unwell
Stridor
Drooling of saliva
Tripod position = easier to breathe if leaning forward and extending in a seated position
282
When should children be able to sit without support?
7-8m
283
Developmental milestones at 3yo
Ride a tricycle using pedals, walks upstairs without holding onto rail
284
S+Sx of dyskinetic cerebral palsy
ATHETOID MOVEMENTS AND ORO-MOTOR PROBLEMS
Slow writhing movements of hands and feet, difficulty holding onto objects
Drooling
285
Following birth of a baby, what should you ensure you do?
1. Dry the baby, maintain temperature, start clock
2. Assess tone, breathing and HR
3. If gasping/not breathing --> give 5 inflation breaths
4. Reassess HR , if no increase ensure adequate breaths are being given by checking chest movement
5. If chest not moving you assume inflation breaths are inadequate --> recheck head position, consider 2-person airway control and repeat inflation breaths and look for response
6. If chest moving but HR undetectable/<60, start chest compression at ratio of 3compressions to 1inflation breaths
7. Reassess HR every 30secs, if slow or undetectable, consider IV access and drugs
286
List 5 acyanotic congenital heart diseases
VSD (increase risk of endocarditis)
ASD
PDA
Coarctation of the aorta
Aortic valve stenosis
287
Koplik spots (small white lesions on buccal mucosa) pathognomonic for...
Measles
288
What is APGAR score?
Assess health of baby, conducted at 1mins and 5mins of life. If the score is low, it is repeated every 10mins.
289
What does the APGAR score suggest?
0-3 = very LOW
4-6 = moderate low
7-10 = good state
290
What is intraventricular haemorrhage?
Haemorrhage that occurs in the ventricular system of the brain. Occurs in premature neonates spontaneously. The blood may clot and occlude CSF flow and result in hydrocephalus.
291
Tx of intraventricular haemorrhage
Largely supportive
Hydrocephalus and rising ICP: indication for shunting
292
When does transient tachypnoea of newborn usually settle?
Within 1-2days
293
Name a late complication of Down's syndrome
AD
294
List three key Sx of bronchiolitis
Mild fever, persistent cough, wheeze
295
Mx of achondroplasia
No specific therapy. Some benefit from limb lengthening procedures.
296
RFs of achondroplasia
70% sporadic mutation
Advancing parental age at the time of conception
297
Pattern of inheritance of achondroplasia
Autosomal dominant
298
ALL breech babies at/after 36wks require what screening?
DDH - ultrasound scan of the pelvis
299
What murmur is found in TOF?
Ejection systolic murmur due to pulmonary stenosis
300
Explain what the phenomenon of anticipation means in Huntington's disease
Earlier onset and increased severity of disease
301
List 3 S+Sx of androgen insensitivity syndrome
Primary amenorrhoea
Undescended tests causing groin swellings
Breast development may occur as a result of conversion of testosterone to oestradiol
302
What is androgen insensitivity syndrome?
X-linked recessive condition due to end-organ resistance to testosterone causing a genotypically 46XY to have a female phenotype.
303
Mx of androgen insensitivity syndrome
Counselling: raise child as female
Bilateral orchidectomy (increased risk of testicular Ca due to undescended testes)
Oestrogen therapy
304
Ix for androgen insensitivity syndrome
Buccal smear or chromosomal analysis to reveal 46XY genotype
305
When is MenB vaccination given?
2m, 4m and 12-13m
306
What are venous hums?
Continuous blowing noise heard just below the clavicles due to turbulent blood flow in the great veins returning to the heart.
307
Surgical Mx of biliary atresia
The only DEFINITIVE TX
Hepatoportoenterostomy (aka Kasai portoenterostomy) = blocked bile ducts are removed and replaced with a segment of the small intestine. Restores bile flow from liver to proximal small bowel.
If there are signs of end-stgae disease/progressive cholestasis/hepatocellular decompensation, development of severe portal HTN.
308
Mx of scarlet fever
Oral penicillin V 10 days
Note: if allergy to penicillin, give azithromycin
309
S+Sx of neonatal sepsis
Resp distress (85%): grunting, nasal flaring, use of accessory resp muscles, tachypnoea
Tachycardia
Apnoea, jaundice, change is mental status/lethargy
310
S+Sx of benign rolandic seizures
Night-time, partial seizures (possible secondary generalisation), otherwise normal
311
Name a neck mass that rises on protrusion of the tongue
Thyroglossal cyst
312
Cardiac abnormalities in Turner's syndrome
Ejection systolic murmur from bicuspid aortic valve
Coarctation of the aorta
313
Mneumonic for Kawasaki disease presentation
Need at least 5 of the Sx (also more common in <5yo)
CRASH BURN
Conjunctival infection
Rash
Adenopathy (lymph)
Strawberry tongue
Hands (swelling/erythema of hands/feet)
BURN - fever >5days
314
Mx of pyloric stenosis
Ramstedt pyloromyotomy
315
Child presenting with acute limp <3yo
Refer for urgent paediatric assessment to rule out trauma, septic arthritis
316
Ix for Hirschprung's disease
Full-thickness rectal biopsy
317
Limp + fever in a child Mx
Must differentiate between septic arthritis and other serious causes
--> fever is a RED flag, so need urgent specialist assessment
--> if child is 3-9yo, well, febrile, limping, Sx<72hrs, then can be monitored in primary care
318
When is DMSA used?
Assess renal scarring due to vesicoureteric reflux
319
Bronchiolitis vs VIW
Bronchiolitis: less than 12m old, wheeze + FINE CRACKLES
VIW: 1-7yo, wheeze
Asthma is usually >5yo
320
List 5 red flags in a child with a fever
Moderate/severe chest wall recession
Does not wake if aroused
Reduced skin turgor
Mottled/blue appearance
Grunting
321
Why is IV amoxicillin given to babies with cefotaxime?
To cover for Listeria
322
Most common location of urethral opening in boys suffering from the condition?
On the distal ventral surface of the penis
323
Define nephrotic syndrome
Triad of proteinuria (>1g/m2 per 24hrs)
Hypoalbuminaemia (<25g/L)
Oedema
324
Most common cause of nephrotic syndrome in children
80% due to minimal change glomerulonephritis
325
When should you consider prostaglandin E1 for PDAs?
This works differently to indomethacin/ibuprofen as it keeps the patent duct open.
It is used whilst awaiting surgery if PDA is associated with another congenital heart defect amenable to surgery.
326
Ix of choice for intersussception
USS - may show target like mass
327
Define retinoblastoma
Malignant tumour of retinal cells, accounts for 5% of severe visual impairment of children
328
S+Sx of retinoblastoma
Absence of red reflex, replaced by white pupil (leukocoria) - most common
Stabismus
Visual problems
329
Mx of retinoblastoma
Enucleation
For advanced disease: chemotherapy (bilateral) + laser treatment to retina ± chemotherapy (advanced disease)
330
Cause of retinoblastoma
Unilateral (80% spontaneous), bilateral (100% hereditary)
AD, chromosome 13 encoding pRB (protein retinoblastoma)
331
Average age of Dx of retinoblastoma
18m
332
Prognosis of retinoblastoma
Most cured, some may be visually impaired (>90% survive to childhood)
Risk of secondary malignancy (sarcoma) in survivors of hereditary retinoblastoma
333
Define central cyanosis
Conc of reduced Hb in blood exceeds 5g/dl
334
What is the nitrogen washout test?
Used to differentiate cardiac from non-cardiac causes
1. Give infant 100% O2 for 10mins
2. Take ABG
3. pO2 <15kPa >> cyanotic congenital heart disease
335
List 3 causes of cyanotic congenital heart disease
Tetralogy of Fallot (TOF)
Transposition of great arteries (TGA)
Tricuspid atresia
336
Initial Mx of cyanotic congenital heart disease (2)
1. Supportive care
2. Prostaglandin E1 e.g. alprostadil - used to maintain PDA if the congenital heart defect depends on the patent duct
337
Define acrocyanosis
Peripheral cyanosis around mouth and extremities (hands and feet) in healthy newborns persists 24/48hrs
338
Child can walk unsupported by...
13-15m
339
List 5 causes of nappy (napkin) rash
Irritant dermatitis - most common cause, due to irritant effect of urinary ammonia and faeces, spares creases
Candida dermatitis - erythematous rash with involve the flexures and characteristic satellite lesions
Seborrhoeic dermatitis - erythematous rash with flakes, may coexist with scalp rash
Psoriasis - erythematous scaly rash but rare cause
Atopic eczema - extensor
340
General Mx of nappy rash
Disposable nappies are preferable to towel nappies
Expose napkin area to air when possible
Apply barrier cream (e.g. Zinc and castor oil)
Mild steroid cream (1% hydrocortisone) in severe cases
341
Mx of suspected candidal nappy rash
Topical imidazole
Stop the use of barrier cream until candida has settled
342
Define Ebstein's anomaly
Congenital heart defect characterised by low insertion of tricuspid valve resulting in large atrium
343
What is in the Kocher criteria to differentiate between transient synovitis and septic arthritis?
Temp >38.5
Unable to weight-bear on affected limb
ESR > 40
WCC > 12
(very low, 3%, 40%, 93%, 99%)
344
When is it 'normal' to have neonatal hypoglycaemia?
First 24hrs of life, but does not have any negative consequences as they can utilise alternate fuels like ketones and lactate
345
List 2 conditions associated with hypospadias
Cryptorchidism and inguinal hernia
346
5 steps of newborn resus
1. Dry baby and maintain temp
2. Assess tone, RR, HR
3. If gasping/ not breathing give 5 inflation breaths
4. Reassess (chest movements)
5. If HR not improving and <60bpm start compression and ventilation (3:1)
347
State the risk of further febrile convulsions and RFs
1 in 3
RFs for further seizures : age of onset <18m, fever <39, shorter duration of fever before seizure, FHx febrile convulsions
348
What is the risk of child developing epilepsy if they have had a febrile convulsion?
RFs for developing epilepsy: FHx, complex febrile seizures, background of neurodevelopmental disorder
Children with NO RFs: 2.5% risk
Children with all 3 RFs: higher risk of 50%
349
How to manage a child with a limp + fever?
Even if the Dx of transient synovitis is likely, fever is a red flag --> need urgent specialist assessment
350
Define ITP (IDIOPATHIC THROMBOCYTOPENIC PURPURA)
Immune-mediated reduction in platelet count
351
S+Sx of ITP
Bruising
Petechial/ purpuric rash
Bleeding is less common and typically presents as epistaxis/gingival bleeding
352
Ix for ITP (3)
FBC: isolated thrombocytopenia
Blood film
Bone marrow examinations only required if there are atypical features e.g. lymph node enlargement/splenomegaly, high/low WCC, failure to resolve/respond to Tx
353
What do patients with ITP tend to develop?
Viral infection
354
Mx of ITP (3)
1. Usually no Tx required (resolves in 80% of children with 6m +/- Tx)
2. Advice to avoid activities that may result in trauma e.g. team sports
3. If platelet count is very low (<10x10^9/L)/ significant bleeding: oral/IV CS, IV Ig, platelet transfusion used in emergency (e.g. active bleeding) but are only a temporary measure as they are soon destroyed by circulating Abs
355
List 5 conditions that result in obesity in children
GH def
Hypothyroidism
Down's syndrome
Cushing's syndrome
Prader Willi syndrome
356
List the consequences of obesity in children
Orthopaedic issues: slipped upper femoral epiphyses, Blount's disease, MSK pain
Psychological: poor self-esteem, bullying
Sleep apnoea
Benign intracranial HTN
Long-term consequences: increased incidence. ofT2DM, HTN, IHD
357
When should you suspect whooping cough?
Acute cough lasting more than 14days + without another apparent cause and has 1/+ of these features: paroxysmal cough, inspiratory whoop, post-tussive vomiting, undiagnosed apnoeic attacks in young infants
358
Name 3 Abx for whooping cough and its indication
Oral macrolide (e.g. clarithromycin, azithromycin, erythromycin) indicated if cough is within the previous 21 days to eradicate the organism and reduce spread
359
List 2 conditions for which throat Ex is contraindicated
Croup and acute epiglottitis
360
Define juvenile idiopathic arthritis (JIA)
Arthritis occurring in <16yo that lasts for >6wks
361
S+Sx of JIA (7)
Pyrexia
Salmon-pink rash
Lymphadenopathy
Arthritis, uveitis
Anorexia, weight loss
362
Ix for JIA
ANA positive , esp in JIA
Rheumatoid factor negative
363
Difference between early and late shock
Early shock: normal BP, tachycardia, tachypnoea, pale/mottled, reduced
Late shock: hypotension, bradycardia, acidotic (Kussmaul), blue, absent
364
Typical presentation of laryngomalacia
Congenital abnormality of the larynx, very common benign cause of noisy breathing infants
Stridor at 4wks of age
365
Vaccinations given 13-18yo
3 in 1 teen booster (tet, diphtheria, polio)
Men ACWY
366
Venous hums
Innocent murmur, due to turbulent blood flow in great veins returning to heart - continuous blowing noise heard below clavicles
367
Still's murmur
Innocent murmur, low-pitched sound heard at lower left sternal edge
368
List characteristics of an innocent ejection murmur
Soft-blowing murmur in pulmonary area/ short buzzing in aortic area
May vary with posture
Localised with no radiation
No diastolic component, thrill, added sounds (clicks)
Asymptomatic
No other abnormality
369
What organism acute epiglottitis?
Haemophilus influenzae type B
370
Where can lesions of hand, foot, mouth disease be found?
Lesions on hands and feet, groin/buttocks
371
Characterise the rash found in hand, foot. andmouth disease
25mm scattered erythematous macules and papules, often with central greyish vesicle
372
State the 4Ts and 4Hs - reversible causes of cardiac arrest
Hypoxia, hypothermia, hypovolaemia (hypotension), hypo/hyper - kalaemia, glycaemia, calcaemia
Tension pneumothorax, tamponade, thromboembolism, toxicity
373
What is defined as a moderate asthma attack in children 2-5 and >5yo?
SpO2 > 92%
+ in >5yo, PEF >50%
No clinical features of severe asthma
374
Mx of mild-moderate asthma attack in children
Bronchodilator therapy: beta-2-agonist via spacer (for child <3yo, use close-fitting mask), give 1 puff every 30-60secs up to a max of 10puffs, one puff every 30-60secs up to max 10 puffs, if Sx not controlled, repeat beta-2-agonist and refer to hospital
All asthma exacerbation: oral prednisolone (steroid) for 3-5days (20mg od // 30-40mg od)
375
List three characteristics of children associated with Perthes disease
Short, hyperactivity (disruptive behaviour), primary school kids
376
When is the first MMR vaccine given?
12-13m
Then 3-4yo
377
First sign of puberty in males
Testicular growth at around 12yo (10-15yo), >4ml of testicular volume indicates puberty
378
First sign of puberty in females
Breast development around 11.5yo (9-13yo)
379
Immunisations given at 12-13m
Hib/MenC, MMR, PCV, Men B
380
What is CHD associated with?
Pulmonary hypoplasia (as part of a sequence alongside hernial development rather than direct compression of lungs by the herniated viscera)
381
Define pulmonary hypoplasia
Newborn infants with underdeveleped lungs
382
List 2 causes of pulmonary hypoplasia
Oligohydraminos, CHD
383
Mx of umbilical hernias
Typically resolve by 4-5 years of age
If large/symptomatic perform elective repair at 2-3yo
If small and asymptomatic, perform elective repair at 4-5yo
384
List 3 factors associated with umbilical hernias
Afro-Caribbean infants
Down's syndrome
Mucopolysaccharide storage disease
385
Ix of choice for intussusception
Ultrasound scan
386
List 6 S+Sx of cow's milk protein intolerance
Regurg, vomiting
Diarrhoea
Urticaria, atopic eczema
Colic Sx: irritability, crying
Wheeze, chronic cough
Rarely angioedema, anaphylaxis may occur
387
Ix for cow's milk protein intolerance
Usually clinical (cow's milk protein elimination)
Others: Skin prick/patch testing, total IgE and specific IgE RAST for cow's milk protein
388
Mx if severe cow's milk protein intolerance Sx (failure to thrive)
Refer to a paediatrician
389
Mx of cow's milk protein intolerance if formula-fed
Extensive hydrolysed formula (eHF) milk is the first-line replacement formula for infants with ild-moderate symptoms
Amino acid-based formula (AAF) in infants with severe CMPA or if no response to eHF
Around 10% of infants are also intolerant to soya milk
390
Mx of cow's milk protein intolerance if breastfed
Continue breastfeeding
Eliminate cow's milk protein from maternal diet. Consider prescribing calcium supplements for breastfeeding mothers whose babies have, or are suspected to have, CMPI, to prevent deficiency whilst they exclude dairy from their diet
Use eHF milk when breastfeeding stops, until 12 months of age and at least for 6 months
391
Prognosis of CMPR
In children with IgE mediated intolerance around 55% will be milk tolerant by the age of 5 years
In children with non-IgE mediated intolerance most children will be milk tolerant by the age of 3 years
A challenge is often performed in the hospital setting as anaphylaxis can occur
392
List key 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
393
Diagnostic Ix for pyloric stenosis
US Abdomen
394
Presentation of laryngomalacia
Congenital abnormality of the larynx. Infants typically present at 4wks of age with stridor.
395
List 4 DDx of stridor
Croup
Acute epiglottitis
Inhaled foreign body
Laryngomalacia
396
What is transient synovitis referred to as?
Irritable hip
397
List 6 Ix for fever in <3m
FBC
Blood culture
CRP
Urine test for UTI
CXR if resp signs present
Stool culture if diarrhoea present
398
Describe the rash that presents with Parvovirus B19
Slapped cheek syndrome, where the rash starts on the cheeks and then spreads
399
What electrolyte disturbance is caused by pyloric stenosis?
Hypochloraemic, hypokalaemic metabolic alkalosis
400
RFs of meconium aspiration syndrome
Post-term born after 42wks (44%)
Maternal HTN, pre-eclampsia, chorioamnionitis, smoking, substance abuse
401
School exclusion criteria for scarlet fever
24hrs after starting Abx
402
True or false: ITP is often preceded by viral illness
True
403
If a baby fits the screening criteria for DDH, when should they be screened using USS?
6wks
404
Pain after exercise, intermittent swelling and locking of knees
Osteochondritis dissecans
A joint condition in which bone underneath the cartilage of a joint dies due to lack of blood flow. This bone and cartilage can then break loose, causing pain and possibly hindering joint motion.
405
First sign of puberty in girls
Breast development around 11.5yo of age (9-13)
406
RR and HR of healthy infants
RR of 30-60 breaths per min
Regular pulse of 100-160bpm
407
Murmur for VSD
Pansystolic murmur in lower left sternal border
408
Murmur for coarctation of aorta
Cresendo-decrescendo murmur in upper left sternal border
409
Murmur for PDA
Diastolic machinery murmur in the upper left sternal border
410
Murmur for pulmonary stenosis
Ejection systolic murmur in upper left sternal border
411
Mx of Perthes disease
Keep femoral head within acetabulum: cast, braces
If less than 6yo, observation
Older: surgical Mx with moderate results
Operate on severe deformities
412
Prognosis of Perthes disease
Most cases resolve with conservative Mx, early Dx improves outcomes.
413
When should you admit the child for bronchiolitis?
Usually self-limiting, but important to deteriorate in the first 72hrs of illness:
Apnoea, persistent O2 sats of <92%
Inadequate fluid intake (<50% of normal fluid intake), persisting severe resp distress e.g. grunting, marked chest recession, resp rate >70breaths/min
414
Murmur for bicuspid aortic valves
Ejection systolic murmurs
415
Organism accountable for croup?
Parainfluenza virus
416
Organism accountable for bronchiolitis?
RSV
417
Organism accountable for whooping cough
Bordetella pertussis
418
What is transposition of the great arteries?
Aorta leaves the right ventricle, pulmonary trunk leaves the left ventricle
419
S+Sx of transposition of the great arteries
Cyanosis, tachypnoea, loud single S2, prominent right ventricular impulse, egg-on-side appearance on CXR
420
Mx of transposition of the great artiers
Maintenance of the ductus arteriosus with prostaglandins, surgical correction is definite
421
Immediate Mx of CDH in a neonate
Immediately intubate and ventilate
422
Mx of immune thrombocytopenia (idiopathic thrombocytopenia)
Usually no Tx required. Normally resolves in around 80% of children with 6m. If platelet count is <10x10^9/L / sig bleeding, then oral/IV CS or Ig, platelet transfusion used in an emergency but only a temp measure as they are soon destroyed by circulating Abs.
423
Hand preference abnormality
Hand preference before age of 12m >> cerebral palsy
424
Name the hearing test conducted at >3yo
Pure tone audiometry
425
Mx of UTI in >3m
Treat with oral Abx for 3days according to local guidelines, usually trimetoprim, niturofurantoin, cephalosporin, amoxicillin
426
Key features of Osgood-Schlatter disease
Seen in sporty teens. Pain, tenderness and swelling over tibial tubercle.
427
Cause of roseola infantum
HHV6
428
List features of atypical UTIs
Seriously ill, poor urine flow, abdo/bladder mass, raised Cr, septicaemia, failure to respond to Tx with suitable Abx within 48hrs, infection with non-E.coli organisms
429
Pattern of inheritance of hereditary haemochromatosis
Autosomal recessive
430
List 3 features of Pierre-Robin syndrome
Micrognathia, posterior displacement of the tongue (may result in upper airway obstruction), cleft palate
431
When do you give oral macrolide for whooping cough?
If the onset of cough is within previous 21days to eradicate the organism and reduce the spread
432
When is rotavirus vaccine given?
2 doses - 2m and 3m
433
When should you not give rotavirus?
1st dose after 14+6
2nd dose 23+6 due to theoretical risk of intussusception
434
Classic presentation of chickenpox
Increased temp of 2days before developing erythematous vesicles affecting predominantly the torso and face
435
When is the infectious period of chicken pox?
1-2days before the rash appears, infectivity continues until all the lesions are dry and have crusted over (5days after onset of rash)
436
Mx of a child below 0.4th centrile for height
Referral to paediatric outpatients clinic
437
Mx of a child below 2nd centile for height
Review by GP
438
Mx of CF patients
MDT approach
1. Regular (at least 2 times a day) chest physiotherapy and postural drainage, deep breathing exercises
2. High calorie diet, high fat intake with pancreatic enzyme supplementation for every meal
3. Minimise contact with each other to prevent cross infection with Burukholderia cepacia complex and pseudomonas aeruginosa
4. Vitamin supplementation
5. Lung transplantation - CI in patients with crhonic Burkholderia cepacia
439
Triad of features of Shaken Baby Syndrome
Retinal haemorrhages, subdural haematoma, encephalopathy
440
When are bone marrow ex required for ITP?
When there are atypical features e.g. lymph node enlargement/splenomegaly, high/low WCC, failure to resolve/ respond to Tx
441
When is the heel prick test conducted?
5 and 9 days of life
442
What causes CROUP?
Parainfluenza VIRUS
443
Most common complication of roseola infantum
FEBRILE CONVULSIONS
444
Mx for child presenting with ?transient synovitis
Urgent paeds referral for assessment
445
Characteristics of toddler's diarrhoea
Stool varying in consistency, may contain undigested food
