Paediatrics Flashcards
1
Q
What are the organisms causing newborn pneumonia?
A
Group B streptococcus from mother’s genital tract
Gram-neg enterococci
2
Q
What organisms cause pneumonia in infants and young children?
A
Respiratory viruses (RSV in particular)
Streptococcus pneumonia
Haemophilus influenza
Bordetella pertussis
3
Q
What organisms cause pneumonia in children over 5
A
Mycoplasma pneumoniae
Streptococcus pneumoniae
Chlamydia pneumoniae
4
Q
What are the classic signs of consolidation?
A
Dullness on percussion
Decreased breath sounds
Bronchial breathing over the affected area
(these are often absent in young children)
5
Q
What is the antibiotic of choice for pneumonia
A
Amoxicillin or erythromycin for over 5 year olds
Co-amoxiclav if complicated or unresponsive
6
Q
What is the defect in cystic fibrosis
A
Defective protein in the cystic fibrosis transmembrane conductance regulator (CFTR) on chromosome 7
7
Q
What is CFTR
A
A cyclic AMP-dependent chloride channel
8
Q
What is the pathophysiology of CF?
A
Abnormal ion transport across epithelial cells
9
Q
What are the other complications of CF apart from resp?
A
Meconium ileus
Blocks pancreatic ducts - pancreatic enzyme deficiency and malabsorption
Abnormal sweat gland function
10
Q
What is the diagnostic test for CF
A
The sweat test - high chloride concentration
And gene testing
11
Q
What is the most common causative organisms for peri orbital cellulitis
A
Staphylococcus Aureus
Group A strep
Haemophilus Influenzae
12
Q
What should be excluded in diagnosis of a squint
A
Retinoblastoma - check red reflexes
Cataracts
13
Q
After how many months old should a baby with a squint be referred to a specialist?
A
3 months - newborn babies normally have transient misalignments
14
Q
What are the two types of squints?
A
Concomitant (usually due to a refractive error in one eye)
Paralytic (sinister causes such as a space-occupying lesion)
15
Q
How to test for a squint with a pen-torch
A
Red reflex in both eyes at the same time - if not, there is a squint
16
Q
Severe GORD is more common in children with what?
A
Cerebral palsy and neurodevelopment disorders
Preterm infants
Following surgery fro oesophageal atresia or diaphragmatic hernia
17
Q
Complications of GORD
A
Failure to thrive due to severe vomiting
Oesophagitis
Recurrent pulmonary aspiration
Dystonic neck posturing
Apparent life-threatening events (ALTE)
18
Q
How is failure to thrive defined on a growth chart?
A
Mild: a fall across 2 centile lines
Severe: a fall across 3 centime lines
19
Q
Causes of failure to thrive
A
Inadequate intake
Inadequate retention
Malabsorption
Failure to utilise nutrients
Increased requirements
20
Q
What is marasmus
A
Weight for height 3 standard deviations below the median and a wasted, wizened appearance
Skin-fold appearance and id-arm circumference markedly reduced
21
Q
What is Kwashiorkor
A
Severe protein malnutrition with generalised oedema as well as severe wasting. Weight may not be reduced due to oedema
Can happen when infants are weaned from the breast quite late or if diet is high in starch
22
Q
Features of Kwashiorkor
A
‘Flaky-paint’ skin rash with hyper keratosis and desquamation
Distended abdomen and enlarged liver
Angular stomatitis
Hair which is sparse and depigmented
Diarrhoea, hypothermia, bradycardia and hypotension
Low plasma albumin, potassium, glucose and magnesium
23
Q
Management of severe acute malnutrition
A
Hypoglycaemia
Hypothermia
Dehydration (but avoid being overzealous with IV fluids as can lead to heart failure)
Electrolytes
Infection
Micronutrients
24
Q
What is Hirschprung disease
A
The absence of myenteric plexuses of rectum and variable distance of colon. Causes a narrow, contracted segment
25
How does Hirschsprung disease present
Intestinal obstruction in the newborn period following delay in passing meconium. Or in later childhood - profound chronic constipation, abdominal distension and growth failure. Digital rectal exam can cause a release of stool and flatus
26
How does Meckel diverticulum present
Generally asymptomatic, but may present with bleeding, intussusception, volvulus or diverticulitis
27
What is biliary atresia
Destruction or absence of the extra hepatic biliary tree and intra-hepatic biliary ducts. Untreated, it leads to chronic liver failure and death.
28
What is the surgical management of biliary atresia?
Hepatoportoenterostomy (Kasai procedure) to bypass the fibrosed ducts
29
What are choledochal cysts?
Cystic dilatations of the extra hepatic biliary system
30
How do choledochal cysts present?
In infancy with cholestasis. In older age, present with abdominal pain, a palpable mass, jaundice or cholangitis.
31
How does neonatal hepatitis syndrome present?
Prolonged neonatal jaundice and hepatic inflammation
May have intrauterine growth restriction and hepatosplenomegaly at birth
31
How does neonatal hepatitis syndrome present?
Prolonged neonatal jaundice and hepatic inflammation
May have intrauterine growth restriction and hepatosplenomegaly at birth
32
What can cause neonatal hepatitis syndrome
Alpha1-antitrypsin deficiency
Galactosaenia
+ other causes
33
Causes of acute liver failure in children
Paracetamol overdose
non-A to G viral hepatitis
Metabolic conditions
34
How does acute liver failure in children present?
Jaundice
Encephalopathy
Coagulopathy
Hypoglycaemia
Electrolyte disturbance
35
Investigation findings in acute liver failure
Very high transaminases
Very abnormal coagulation
Elevated plasma ammonia
36
What are the complications of acute liver failure
Cerebral oedema
Haemorrhage from gastritis or coagulopathy
Sepsis
Pancreatitis
37
Management of acute liver failure
Maintain blood glucose - IV dextrose
Prevent sepsis - broad spectrum Abx and antifungals
Prevent haemorrhage - IV vit K, fresh frozen plasma or cryoprecipitate and H2-blocking drugs or PPIs
Treat cerebral oedema - fluid restrict and mannitol diuresis
LIVER TRANSPLANT
38
What medication is Reye syndrome linked to
Aspirin in under 12s
39
What is Reye syndrome
Acute non-inflammatory encephalopathy with micro vesicular fatty infiltration of the liver
40
When do you consider treatment for nocturnal enuresis
After 6 yrs old
41
What is the management for nocturnal enuresis
Star charts
Enuresis alarm
Sometimes desmopressin
42
Pre-renal causes of acute renal failure (most common in children)
Hypovolaemia (D&V, burns, sepsis, haemorrhage, nephrotic syndrome)
Circulatory failure
43
Renal causes of acute renal failure
Vascular (haemolytic uraemia syndrome, vasculitis, embolus, renal vein thrombosis)
Tubular (acute tubular necrosis, ischaemic, toxic, obstructive)
Glomerular (glomerulonephritis)
Interstitial (interstitial nephritis, pyelonephritis0
44
Post-renal causes of acute renal failure
Obstruction (congenital or acquired)
45
What is haemolytic uraemic syndrome?
Triad of:
Acute renal failure
Haemolytic anaemia
Thrombocytopaenia.
Often occurs after an infectious diarrhoea like Shigella
46
Causes of chronic renal failure in children
Structural malformations
Glomerulonephritis
Hereditary nephropathies
Systemic diseases
47
Features of chronic renal failure in children
Anorexia and lethargy
Polydipsia and polyuria
Failure to thrive/growth failure
Bony deformities from renal osteodystrophy (renal rickets)
Hypertension
Acute-on-chronic renal failure (precipitated by infection or dehydration)
Incidental finding of proteinuria
Unexplained normochromic, normocytic anaemia
48
What does acute nephritis lead to:
Decreased urine output and volume overload
Hypertension, which may cause seizures
Oedema, characteristically around the eyes
Haematuria and proteinuria
49
What can cause nephritic syndrome (acute nephritis)?
Post-streptococcal or post-infectious nephritis
Henoch-schönlein Purpura (or other types of vasculitis)
IgA nephropathy and mesangiocapillary glomerulonephritis
Anti-glomerular basement membrane disease (Goodpasture syndrome)
Familial (Alport syndrome. An X-linked recessive)
50
Signs and symptoms of Henoch-Schönlein purpura
Rash - buttocks, extensor surfaces of legs and arms, ankles
Joint pain and swelling
Abdo pain (haematemesis and melaena, intussusception)
Renal (microscopic/macroscopic haematuria, nephrotic syndrome)
51
What is hereditary angioedema
Rare autosomal dominant
No urticaria but subcutaneous swellings with abdominal pain.
Usually triggered by trauma
Can cause resp obstruction
Can be treated in the short term with fresh frozen plasma replacement
52
What is erythema nodosum
Tender nodules over the legs
Often also have a fever and arthralgia
53
Causes of erythema nodosum
Streptococcal infection
Primary TB
IBD
Drug reaction
Idiopathic
54
What is erythema multiforme
Target lesions with a central papule surrounded by an erythematous ring. Lesions may also be vesicular or bullous
55
Causes of erythema multiforme
HSV
56
What is the age range for febrile seizures?
6 months to 6 years old
57
Febrile seizures don't normally increase risk of epilepsy. What types of febrile seizures do?
Focal, prolonged or repeated in the same illness. Need to rule out intracranial infection like meningitis or encephalitis
58
What is the age of onset for West syndrome (epileptic syndrome)
4-6 months
59
What is the age of onset for Lennox-Gastaut syndrome (epileptic syndrome)
1-3 years
60
What is the age of onset for childhood absence epilepsy?
4-12 years
61
What is the age of onset for benign epilepsy, with centrotemporal spikes (BECTS)
4-10 years
62
What is the age of onset for early-onset benign childhood occipital epilepsy?
1-14 years
63
What is the age of onset for juvenile myoclonic epilepsy
Adolescence-adulthood
64
What can absence seizures be induced by?
Hyperventilation
65
What is the prognosis for childhood absence seizures
95% remission in adolescence, 5-10% develop tonic-clonic seizures in adult life
66
What is the treatment for West syndrome (epileptic syndrome)
Corticosteroids or vigabatrin
67
What is the first line drug for focal seizures?
Lamotrigine
68
What two features of development are autistic children grossly delayed in?
Social skills
Communication skills
69
What is the limit age for sitting without support?
9 months (median is 6 months)
70
What is the limit age for pushing up on arms and holding head up
3 months (median is 6 weeks)
71
What is the median age fro cruising around furniture
9-10 months (limit is 13 months)
72
What are the median and limit ages for fixing and following?
6 weeks
3 months
73
What are the median and limit ages for reaching out for toys
4 months
6 months
74
What are the median and limit ages for transferring between hands?
7 months
9 months
75
What are the median and limit ages for pincer grip
10 months
12 months
76
What are the median and limit ages for polysyllabic babble
3-4 months
7 months
77
What are the median and limit ages for indiscriminate consonant babble (dada, mama)
7 months
10 months
78
What are the median and limit ages for saying 6 words with meaning
12 months
18 months
79
What is the limit age for joining words
2 years
80
What is the limit age for 3 word sentences
2 1/2 years
81
What are the median and limit ages for a responsive smile
6 weeks
8 weeks
82
What are the median and limit ages for symbolic play
18-24 months
2-2 1/2 years
83
What are the median and limit ages for interactive play
2.5-3 years
3-3.5 years
84
Features of fractures that are likely to be inflicted
Fracture in an immobile child
Rib fractures
Multiple fractures
Multiple fractures of different ages
85
Features of bruises that are likely to be inflicted
Shape of a hand or object
On neck like strangulation
Wrists or ankles that look like ligature marks
Bruises to the buttocks in a child less than 2 years or any age without a good explanation
86
Features of burns that are likely to be inflicted
Any burn in a child who is not mobile
A burn in the shape of an implement - cigarette, iron
A 'glove or stocking' burn consistent with forced immersion
87
Features of bites that are likely to be inflicted
Bruising in the shape of a bite thought unlikely to have been caused by a young child
88
What investigation should be done in patients you suspect physical abuse fractures?
Full radiographic skeletal survey with oblique views of the ribs if under 30 months of age (2.5 yrs old)
89
What medical conditions should you consider in suspected child abuse?
Coagulation disorders
Osteogenesis imperfecta
Scalds and cigarette burns - may be bullies impetigo or scalded skin syndrome
90
What investigations should be done in suspected brain injury
CT immediately followed by MRI
Skeletal survey
Expert ophthalmological examination to identify retinal haemorrhages
Coag screen
91
What chromosomal abnormality is present in Edward's syndrome
Trisomy 18
92
What chromosomal abnormality is present in Patau's syndrome
Trisomy 13
93
Clinical features of Edwards syndrome
Low birthweight
Prominent occiput
Small mouth and chin
Short sternum
Flexed, overlapping fingers
Rocker-bottom feet
Cardiac and renal malformations
94
Clinical features of Patau's syndrome
Structural defect of brain
Scalp defects
Small eyes(microphthalmia) and other eye defects
Cleft lip and palate
Polydactyly
Cardiac and renal malformations
95
What is the prevalence of fragile X syndrome
1 in 4000
96
What causes fragile x syndrome
CGG trinucleotide repeat expansion
Normal is below 50 repeats
Pre-mutation is 55-199
Full mutation is 200 +
97
Do female carriers of fragile x syndrome experience any symptoms
50% do and have mild to moderate learning difficulties
98
What are some of the characteristic facies of someone with fragile X
Protruding/everted ears
Long face
Prominent mandible
Broad forehead
99
What are the non-facial features of fragile X
Macrocephaly
Macro-orchidism
Mitral valve prolapse, joint laxity, scoliosis, autism, hyperactivity
100
What are the clinical features of Noonan syndrome
Occasional mild learning difficulties
Short webbed neck with trident hair line
Pectus excavatum
Short stature
Congenital heart disease (especially pulmonary stenosis, atrial septal defect)
Characteristic facies
101
What is the inheritance pattern for Noonan syndrome
Autosomal dominant
102
What are the clinical features of Williams syndrome
Short stature
Transient neonatal hypercalcaemia (occasionally)
Congenital heart disease (supravalvular aortic stenosis)
Mild to moderate learning difficulties
Characteristic facies
103
Signs of respiratory distress in a neonate
Tachypnoea (>60 breaths/min)
Laboured breathing, with chest wall recession (particularly sternal and subcostal indrawing) and nasal flaring
Expiratory grunting
Cyanosis if severe
104
Is meconium passed more frequently the more pre term or the more term
The greater the gestational age, affecting 20-25% of deliveries by 42 weeks
105
Meconium causes resp distress in two ways. What are they
Mechanical obstruction
Chemical pneumonitis (it is a lung irritant)
106
What are the chest X-ray signs of meconium aspiration
Overinflation
Patches of consolidation and collapse
High incidence of pneumothorax and pneumomediastinum
May develop PPH of he newborn
107
What is the main complication of diaphragmatic hernia
Pulmonary hypoplasia due to fatal development being prevented (high mortality)
108
What is the most common cause of respiratory distress in term infants
Transient tachypnoea of the newborn - due to delay in resorbing lung liquid, more common after a C-section
109
What can RDS cause in the lungs
PIE pulmonary interstitial emphysema
Or
Pneumothorax due to overdistended alveoli - increased O2 requirement and reduced breath sounds and chest movement. Use lowest pressures possible to prevent pneumothorax.
110
What is bronchopulmonary dysplasia
Chronic lung disease - oxygen still required at post-menstrual age 36 weeks
111
What are the CXR findings in bronchopulmonary dysplasia
Widespread areas of opacification (fibrosis), sometimes with cystic changes
112
Grades of HIE
Mild - irritable infant, responding excessively to stimulation, staring or the eyes, hyperventilation, impaired feeding
Moderate - marked abnormalities of tone and movement, cannot feed and may have seizures
Severe - no normal spontaneous movements or response to pain. Fluctuate from hypotonia to hypertonia, prolonged seizures, multi-organ failure.
113
What does TORCH stand for
Toxoplasmosis
Other agents (parvovirus, VZV, Syphilis)
Rubella
Cytomegalovirus
Herpes simplex Virus
114
What does Rubella cause before 8 weeks gestation
Deafness
Congenital heart disease
Cataracts
(in over 80%)
115
Beyond what gestational age is the risk of Rubella to the foetus minimal?
18 weeks
116
What can congenital cytomegalovirus cause at birth
Hepatosplenomegaly
Petechiae
Most will have neurodevelopment disabilities such as sensorineural hearing loss, cerebral palsy, epilepsy and cognitive impairment
90% are normal at birth
117
How can Toxoplasma gondii be transmitted
Consumption of raw or undercooked meat
Contact with faeces of recently infected cats
118
While rare, what are the clinical features of toxoplasmosis in an infected infant?
Retinopathy, an acute fundal chorioretinitis which sometimes interferes with vision
Cerebral calcificaiton
Hydrocephalus
(long term neurological disabilities)
119
What is the treatment regime for infants infected with toxoplasmosis
Pyrimethamine
Sulfadiazine
1 year
120
What periods in the pregnancy is the foetus at risk if the mother develops chickenpox?
First half of pregnancy (small risk of severe scarring of skin and possible ocular and neurological damage and digital dysplasia)
Within 5 days before or 2 days after delivery - 25% develop a vesicular rash with a mortality as high as 30%
121
While rare, what are the specific clinical features of congenital syphilis
Rash on soles of feet and hands
Bone lesions.
If mother is treated at least 1 month before delivery, foetus protected
122
Clinical features of TORCH infection
Growth restriction
Eye defects
Pneumonitis
Hepatomegaly
Virus in urine
Bone abnormalities
Intracerebral calcification
Hydrocephalus
Microcephalus
Deafness
Heart defects
Splenomegaly
Rash: blueberry muffin or petechial
Anaemia
Neutropenia
Thrombocytopenia
123
What is a risk factor for necrotising enterocolitis in pre terms
Cows milk formula
124
Symptoms of NEC
Infant stops tolerating feeds
Bile-stained vomit
Distended abdomen
Stool sometimes containing fresh blood
Shock
125
Characteristic signs on Xray of NEC
Distended loops of bowel and thickening of the bowel wall with intramural gas
126
What is the treatment for NEC
Stop oral feeding
Broad spectrum antibiotics
Parenteral nutrition
Artificial ventilation and circulatory support
Surgery if perforated bowel
127
What is gastroschisis
Bowel protruding through a defect in the anterior abdominal wall adjacent to the umbilicus with no covering sac
128
What are the risks of gastroschisis
Dehydration
Protein loss
129
What is oesophageal atresia usually associated with?
Tracheo-oesophageal fistula
Polyhydramnios
130
How does oesophageal atresia present?
Drooling and persistent salivation
Coughing and choking when fed and having cyanotic episodes
131
What trisomy is duodenal atresia associated with?
Down's syndrome
132
Why does maternal hyperglycaemia cause macrosomia?
Glucose crosses the placenta but insulin doesn't, causing an increased insulin secretion by the foetus, promoting growth.
133
What foetal problems are associated with maternal diabetes?
Congenital malformations
Intrauterine growth restriction (microvascular disease)
Macrosomia
134
What neonatal problems are associated with maternal diabetes?
Hypoglycaemia
Respiratory distress syndrome
Hypertrophic cardiomyopathy
Polycythaemia
135
When is hypoglycaemia most common in neonates?
First 24hrs in babies with IUGR, maternal diabetes, large-for-dates, hypothermic, polycythaemic or ill
- due to poor glycemic stores or hyperinsulinaemia
136
When does Group-B strep infection present in neonates
Early-onset - respiratory distress and pneumonia as a newborn
Late-onset - meningitis or focal infection up to 3 months old.
137
What causes cleft lip and palate?
failure of fusion of the frontonasal and maxillary processes
138
What are the 8 week immunisations?
6-in-1
Rotavirus
MenB
139
What are the 12 weeks immunisations?
6-in-1 (2nd dose)
Pneumococcal vaccinee (PCV)
Rotavirus (2nd dose)
140
What are the 16 week immunisations?
6-in-1 (3rd vaccine)
MenB (2nd dose)
141
What are the 1 year immunisations?
HiB/MenC (1st dose)
MMR (1st dose)
Pneumococcal vaccine (PCV)
MenB (3rd dose)
142
What are the 3 year 4 months immunisations?
MMR (2nd dose)
4-in-1 preschool booster
143
What is the 12-13 years immunisation?
HPV
144
What are the 14 year old immunisations?
3-in-1 teenage booster
MenACWY
145
What is radio-femoral delay a sign of in neonates?
Coarctation of the aorta
146
What type of murmur does a VSD cause?
Pansystolic murmur at the lower left sternal edge
Doesn't radiate to the back
Second heart sound is normal (unlike the splitting that happens in ASD)
147
What age range for bronchiolitis
Usually under 1 year olds
148
How to manage an unconscious child who has choked
5 rescue breaths mouth-to-mouth then CPR
149
How is malrotation of the bowel diagnosed?
Upper gastrointestinal contrast study
150
Children with Down syndrome have a four-fold increased risk of which malignancy?
Acute lymphocytic leukaemia
151
Under what age is jaundice always pathological
Jaundice in the first 24 hrs is always pathological
152
Why is IM Vit K offered to all newborn babies?
All babies are born Vit K deficient. Can lead to jaundice due to increased bleeding.
153
What are the indications for tonsillectomy in recurrent tonsillitis?
Seven or more episode in 1 year
Five or more episodes/ year for 2 years
Three or more episodes/year for 3 years
154
Which human herpes virus is Varicella Zoster?
Human herpes virus 3
155
Which human herpes virus causes cold sores and genital warts?
Human herpes virus 1/2
156
Which human herpes virus causes roseola (red lace like rash and high fever)
Human herpes virus 6/7
157
Which human herpes virus causes Epstein Barr Virus?
Human herpes virus 4
158
Which human herpes virus causes Kaposi's sarcoma in HIV children?
Human herpes virus 8
159
What investigation do you need to do when suspected diagnosis of Henoch-Schönlein purpura
Urine dipstick to test for renal impairment (can cause nephritis)
160
How does biliary atresia present?
Prolonged jaundice, hepatomegaly and raised conjugative bilirubin level.
161
Features of hydrocephalus
Enlarged head circumference
Features of raised ICP: irritability, lethargy, vomiting, sunsetting of the eyes,
Tense anterior fontanelle
Distended scalp veins
162
Causes of hydrocephalus
Congenital malformation (stenosis of the aqueduct or a Chiari malformation)
Tumour or vascular malformation in the posterior fossa
Intraventricular haemorrhage(in premature infants in particular)
Failure to reabsorb CSF due to injury to arachnoid villi
Meningitis or encephalitis
163
What causes acute chest syndrome in sickle cell disease
Infarction of the lung parenchyma
164
How do you diagnose Hirschsprung's?
Rectal suction biopsy
165
When is the onset for haemolytic anaemia?
from 24hrs of life
166
What is a hallmark sign on an X-ray of duodenal atresia
Double bubble - one gas bubble in the stomach and the other in the proximal small intestine
167
What are the features of rickets?
Bow legs
Harrison's sulci (groove at the lower end of the rib cage seen in children with weak bones or with chronic respiratory disease)
Constipation
168
What are some risk factors for rickets?
Dark skin
Breastfed for a long time
169
What are the features of osteosarcoma?
Pain and swelling with a prolonged onset
Typically occurs in the metaphyses of long bones
170
What are the x-ray features of osteosarcoma
Disorganised bony growth with a poorly defined border
Periosteal reaction causing a sunburst appearance
171
What type of vaccine is the MMR?
Live attenuated
172
Which vaccines are killed pathogens?
RIP
Rabies
Influenza
Polio
173
Which vaccines are conjugate vaccines?
HiB
PCV (pneumococcal conjugate vaccine)
174
Which vaccine is a polysaccharide vaccine?
PPV (pneumococcal polysaccharide vaccine)
175
Which vaccines are toxoid vaccines?
Diphtheria
Tetanus
(molecules similar to bacterial toxins)
176
What are the doses of adrenaline 1:1000 for anaphylaxis in different ages?
150 micrograms if under 6
300 micrograms if 6-12
500 micrograms if above 12
177
Which scan is used to confirm a diagnosis of Meckel's diverticulum?
Technetium scan
178
What is the commonest cause of rectal bleeding in children?
Meckel's diverticulum
179
What is the management for Tetralogy of Fallot spells?
1st line. Lie babies on their back and bend their needs. + oxygen if in hospital
Prophylaxis: propranolol
Last line. Phenylephrine - vasoconstriction increases systemic vascular resistance helping reverse the shunt and improve cyanosis
Mainly need surgery
180
When does breast milk jaundice start and resolve by
Stars in the first 2 weeks
Resolved by 4 weeks old
181
What medication is give for prophylaxis of bronchiolitis?
Palivizumab - monoclonal antibody against RSV
Given monthly subcutaneously
To children with acyanotic heart disease for example (e.g. VSD in Down's)
182
Which syndrome are aortic arch defects associated with?
DiGeorge syndrome
183
Which genetic abnormality causes DiGeorge syndrome?
22q11.2 deletion
184
What congenital heart defects are associated with Turner's syndrome?
Coarctation of the aorta
Bicuspid aortic valves
185
What congenital heart defects are associated with Noonan syndrome?
Pulmonary stenosis
186
What congenital heart defects are associated with Edward's syndrome?
Septal defects
187
What congenital heart defect is associated with Patau syndrome?
Dextrocardia
188
What are the signs of impending respiratory failure in a patient with an asthma attack?
Silent chest
Low RR
Normalising CO2
Respiratory muscles are getting tired
189
What is the first line treatment for croup?
Oral dexamethasone
190
What are the 5 things assessed in APGAR?
Appearance
Pulse
Grimace
Activity
Respiration
191
How do you keep duct patency in a duct dependent congenital heart defect (E.g. Trasnposition of the great arteries)?
Prostaglandins
DON'T give ibuprofen with this because it antagonises the action of prostaglandins
192
What is a possible long term complication of measles infection?
Subacute Sclerosing encephalitis
193
What cardia condition is a rare but serious complication of Kawasaki's disease?
Coronary artery aneurysms
194
How would you monitor a patient with an asthma attack?
Peak expiratory flow and continuous oxygen saturation monitoring
195
What investigation do you need to do if you suspect orbital cellulits?
Contrast CT of orbits, sinuses and brain
196
What is the antibiotic of choice for orbital cellulitis in paediatrics?
IV Co-amoxiclav
197
When should a newborn have an ultrasound of the hip?
Any abnormal screening teste (Barlow or Ortolani)
Breech delivery
First-degree family history
198
What causes a silent chest in asthma?
Severe bronchoconstriction meaning that a wheeze isn't even possible
Life-threatening sign
199
What is the most common cause of febrile seizures
Roseola caused by human herpes virus 6
200
What is a common trigger for orbital cellulits?
Bacterial sinusitis
201
How does preseptal cellulitis present?
Eye is oedematous and erythematous but no visual symptoms.
Like orbital cellulitis, it is often preceeded by bacterial sinusitis
202
What is the commonest feature of Turner's syndrome?
Short stature
only 30% of patients have webbed neck
203
What is the most common malignant bone tumour in children?
Osteosarcoma
204
Where do osteosarcomas often happen?
Distal femur and proximal tibia
205
How does Ewing's sarcoma usually present?
Like an infection with a fever. usually affects the diaphysis of long bones
206
a smooth olive-sized mass, projectile vomiting after feeds in a neonate suggests what?
Pyloric stenosis
207
What test is used to determine whether leg length discrepancy is femoral or tibial?
Galleazi
208
What are the main features of Turner's syndrome?
Shield shaped chest,
Low posterior hairline
Webbed neck
May present with not started periods yet
209
What is the incubation period for chicken pox?
up to 3 weeks
210
What is the triad of symptoms in Henoch Schonlein Purpura?
Arthralgia
Non blanching purpuric rash
Abdo pain
211
What is an important investigation in Henoch Schonlein Purpura?
Urine dip - look for blood and protein. IgA immune complexes can deposit in the kidneys and can cause renal involvement
212
What can precede/trigger transient synovitis (inflammation of the join space)?
Viral upper resp infection
213
What are the features of Slipped Upper Femoral Epiphysis?
Obesity
Lack of internal rotation
Limp
Knee pain
Adolescents
214
What is the investigation for Hirschsprung's disease?
Rectal suction biopsy and histological examination
215
What is the most sensitive view on xray for slipped upper femoral epiphysis?
Frog-leg because slip is usually posterior
216
What is the classic finding on ultrasound in intussuseption
'target' sign with echogenic and hypogenic bands - demostrating the invagination of the bowel
217
Where do you feel for a pulse in Paeds life support in infants vs over 1s
Under 1 - brachial pulse
Over 1 - Carotid
218
What is another name for 6th disease?
Roseola infantum
HHV6
219
What are the features of 6th disease?
Febrile and lethargic for a few days followed by a rose coloured, blanching maculopapular rash that starts on the torso/neck.
220
What is another name for 5th disease
Slapped cheek syndrome
221
What sign on general inspection would be seen in babies with congestive cardiac disease?
Hepatomegaly
222
What are the features of Pierre Robin sequence?
Cleft lip
Retracted tongue
Small jaw
Infants often struggle with feeding and breathing
223
What is Tuberous Sclerosis
Autosomal dominant, neurocutaneous syndrome characterised by cellular hyperplasia, tissue dysplasia, haematomas affecting multiple organs
224
What is an important differential of croup?
Bacterial tracheitis
225
What haematological condition are children with Down's syndrome at increased risk of developing?
ALL
226
Why is it important to admit a patient after an anaphylactic reaction, even if they are well and normal?
Anaphylaxis can often occur in a bi-phasic reaction (4-6 hours apart)
227
What genetic karyotype commonly causes recurrent otitis media?
Turner syndrome
228
What is the honeymoon period in type 1 diabetes?
When there is a period of time where the beta cells in the pancreas still have some function, therefore not requiring lots of exogenous insulin
Children need to be monitored carefully
229
How soon after starting phototherapy for newborn jaundice should the serum bilirubin be checked again?
4-6 hours after
230
What is Perthes disease?
Avascular necrosis of the femoral head in children aged 4-8
231
What can accentuate innocent murmers?
febrile illness
232
At what age should a child be able to use a cup and spoon to feed themselves?
18 months
233
What is Hb SC in sickle cell disease?
Milder form of sickle cell disease
234
What is another name for Slapped Cheek Syndrome?
Erythema infectiosum or fifth disease
235
When do es ALL typically present in children?
Under age 6
236
What is a "starry-sky appearance" on bone marrow biopsy characteristic of ?
Burkitt's Lymphoma
237
What is Burkitt's lymphoma?
A high grade B cell lymphoma. which is common in tropical Africa
238
What are the features of Burkitt's lymphoma on a blood film?
Nucleated red cells
Left shift
Basophilic vacuolated lymphoma cells
239
What are some causes of pathological jaundice in the first 24 hours of life?
Rhesus haemolytic disease
ABO incompatibility
G6PD
TORCH screen
240
What is a positive Brudzinski's sign in meningitis?
Neck flexion triggers involuntary knee/hip flexion
241
What congenital infection can cause aqueductal stenosis leading to congenital hydrocephalus?
Rubella
242
What is the inheritance pattern of DMD?
X-linked recessive
243
What are the two conditions that are X-linked dominant?
X-linked hypophosphateaemic rickets
Alport syndrome
244
How long is slapped cheek infectious for?
Until the rash appears
245
Until what age do you use correction for gestational age in development of ex-prem children?
2 years old
246
What is the most common cause of persisting proteinuria in children?
Orthostatic proteinuria
Increases after being on feet for a while
247
What is a Branchial cyst?
Embryological remnant from the development of the branchial arches which form parts of the head and neck
Manifests as a painless cystic mass anterior to the sternocleidomastoid muscle
Presents in late adolescence and doesn't move on swallowing
Anechoic mass on USS
248
What is the most common cause of LATE-onset neonatal sepsis (after 72 h of life)?
Staph aureus
249
What is the most common cause of neonatal jaundice with conjugated hyperbilirubinaemia?
Biliary atresia (surgically treated by the Kasai procedure)
250
What do you give to keep the ductus arteriosus open?
Prostaglandins - Alprostadil (Prostaglandin E1)
251
How is vesicoureteric reflux diagnosed?
Micturating cystourethrogram.
Should be postponed until 3 months after a UTI
252
What is a DMSA scan?
Used to identify scarring of renal tissue
Less specific than a MCUG
Should be taken 3 months after the UTI has resolved
253
What do investigations show in G6PD?
Haemolysis (normocytic anaemia, high reticulocyte count, high LDH) and the blood film may show Heinz bodies and bite cells
254
How is thalassaemia diagnosed?
Haemoglobin electrophoresis
255
Why aren't SSRIs used commonly in the elderly?
SSRIs are associated with increased risk of bleeding when prescribed with anticoagulants
256
What electrolyte upset is commonly seen as a complication in bronchiolitis caused by RSV?
Hyponatraemia
257
What is the investigation for Perthes' disease?
X ray
258
What food allergies are associated with eczema?
Egg, soy, gluten, nuts and fish allergies
259
What are the symptoms of a varicocele?
Usually asymptomatic but can cause a dull discomfort
260
What are some risk factors for cerebral palsy?
Birth complications
Maternal infections
Maternal thyroid dysfunction
Prematurity
261
Is IV sodium bicarbonate used in children with DKA?
No only considered in adults with a pH greater than 6.9
262
What are some side effects of corticosteroids?
Acne, striae, telangiectasia, skin thinning
