Paediatrics Flashcards
1
Q
What is the most common defect in hereditary spherocytosis?
- iron deficiency
- spectrin deficiency
- folic acid deficiency
- vitamin D deficiency
A
- spectrin deficiency
2
Q
What crises are associated with hereditary spherocystosis? (2)
A
- haemolytic crisis
- aplastic crisis
3
Q
8 y.o. pain in R knee
Pain started spontaneously 2 days ago, no history of trauma
Increased pain during weight-bearing
Feels otherwise well, no fever
Exam: appears well at rest, normal BMI, vital signs
Gait: limb
Exam R knee: unremarkable
R hip: reduced range of motion particularly internal rotation + abduction
What is the most likely diagnosis?
- slipped upper femoral epiphysis (SUFE)
- Perthes disease
- iliotibial tract syndrome
- septic arthritis
- patellofemoral pain syndrome
A
- Perthes disease
(SUFE - more common in obese children)
4
Q
6 y.o boy
2 day history of limping, severe pain in L leg whenever weight bears
Several infections recently
Fever over last week and general fatigue
No other PMHx, is up to date with his immunisations and takes no regular medication
Clinical examination: pallor, generalised lymphadenopathy + splenomegaly, significant tenderness over the mid-shaft of the L femur on palpation, but normal range of motion in all joints and an antalgic gait. Vital signs reveal fever and tachycardia
- slipped upper femoral epiphysis (SUFE)
- Perthes disease
- iliotibial tract syndrome
- septic arthritis
- acute lymphoblastic leukaemia (ALL)
A
- acute lymphoblastic leukaemia (ALL)
5
Q
9 y.o. boy
2 day history of pain in R low abdomen
Viral infection last week
Bowel habit is normal and no vomiting
On exam: mild lower abdo tenderness in R iliac fossa, with no associated guarding. No palpable abdom masses
Cervical + submandibular lymphadenopathy and inflmaed tonsils with no exudate
Vital signs are unremarkable
WBC and CRP are only mildly elevated
Urine dip unremarkable
Abdominal USS - unremarkable
- appendicitis
- mesenteric lymphadenitis
- abdominal migraine
- Crohn’s disease
- Intussuception
A
- mesenteric lymphadenitis
(got to rule out appendicitis first)
6
Q
6 y.o. boy admitted on paediatric ward
7 days history of high fevers and irritability
Mouth-sore and desquamated, with ‘strawberry tongue’ appearance
Feet noted oedematous and red
Polymorphous rash widespread
Which medication is most likely to reduce the risk of complications associated with this disease?
- ibuprofen
- amoxicillin
- IV immunoglobulin
- paracetamol
- dexamethasone
A
- IV immunoglobulin
(diagnosis: Kawasaki disease)
7
Q
Most serious complication of Kawasaki disease?
A
= coronary artery aneurysms
8
Q
Why is Aspirin givin in Kawasaki disease?
A
= protect against coronary artery aneurysms
9
Q
4 y.o. in ED
Has had a runny nose and a strange noisy/ barking cough for a few days
This evening breathing became more laboured
She has a RR of 35/ min, SpO2 of 96% on room air, a pulse of 95 bpm and a temp of 37.7oc
On exam: mild intercostal recession and mildly reduced air entry on ausculatation
What is the most appropriate initial management?
- obtain IV access
- nebulised adrenaline
- penicillin V
- nebulised salbutamol
- oral dexamethasone
A
- oral dexamethasone
10
Q
Most common cause of croup?
A
Viral - parainfluenza
11
Q
When do you expect a child to sit up supported?
A
= approx. 6 months
12
Q
What is acute epiglottitis?
A
= rapidly progressive infection that leads to inflammation of the epiglottis and adjacent tissues
13
Q
Cause of acute epiglottitis?
A
= Haemophilus influenza B (Hib)
14
Q
How is acute epiglottitis diagnosed?
A
= direct visualisation of the inflamed epiglottis - typically done using laryngoscopy after securing airway
(do not examine or upset child without senior support)
15
Q
Management of acute epiglottitis? (2)
A
- secure airway, possibly through endotracheal intubation (as first priority)
- administer IV antibiotics, typically cefuroxime
16
Q
What is an APGAR score?
A
= is a method of assessing the state of a neonate quickly after birth
The score is intended as a quick assessment of current status, and is not a long-term prognostic indicator
17
Q
What components make up the APGAR score? (5)
A
A - appearance
P - pulse
G - grimace
A - activity
R - respiration
18
Q
When does NICE adivse to calculate the APGAR score? (2)
A
- 1 minute, AND
- 2 minutes of life
19
Q
Most common cancer in children?
A
= acute lymphocytic leukaemia (ALL)
20
Q
What is acute lymphocytic leukaemia (ALL)?
A
= a malignant condition that arises from the uncontrolled proliferation of genetically altered lymphoid progenitor cells
21
Q
How is acute lymphocytic leukaemia (ALL) definatively diagnosed?
A
= bone marrow biopsy
22
Q
Acute exacerbation of asthma can be triggered by? (4)
A
- Allergens: dust pollution, animal hair, smoke
- Respiratory infections: viruses such as common cold
- Exercise: especially in cold weather
- Emotional stress: can lead to hyperventilation and symptoms of an asthma attack
23
Q
Signs of severe acute episode of asthma? (3)
A
- Respiratory distress – use of accessory muscles of respiration, breathlessness resulting in inability to complete sentences, tachypnoea with a RR > 30/min if over 5, >40 if under 5
- HR > 125/min if over 5, >140/min if under 5
- Peak expiratory flow rate 33-50% of predicted
24
Q
Life-threatning features of acute exacerbation of asthma in a child? (7)
A
- peak expiratory flow rate < 33% predicted
- oxygen saturations < 92%
- silent chest on auscultation
- weak or no respiratory effort
- hypotension
- exhaustion
- confusion/ altered conscious level
25
What is acute otitis media?
= a prevalent infection that leads to inflammation of the middle ear
26
Primary cause of otitis media?
= bacterial infection
27
When would you admit a child with otitis media? (2)
- if < 3 monhts with temp of 38, OR with suspected complications
- consider if systemically unwell
28
What can be used to treat pain + fever in acute otitis media? (2)
- parcetamol
- ibuprofen
29
Anaemia: causes of reduced red cell production? (6)
- iron deficiency
- folic acid deficiency
- B12 deficiency
- red cell aplasia
- chronic renal failure
- chronic inflammation
30
Anaemia: causes of increased erythrocyte destruction? (5)
- G6PD deficiency
- hereditary spherocytosis
- sickle cell disease
- thalassemia
- haemolytic disease of the new-born
31
What type of hypersensitivity reaction is anaphylaxis?
= type I hypersensitivity reaction
32
Primary investigation in paediatric anaphylaxis?
= serum levels of mast cell tryptase
(rises within 1 hour of onset + can confirm diagnosis)
33
What position is best to put a child in anaphylaxis?
= supine position and raise their legs
34
How long should paediatric patients be monitored post-anaphylaxis? why?
= 6-12 hours, in case of rebound episode
35
How many adrenaline auto-injectors are given to newly diagnosed paediatric patients after an anphylaxis episode?
= 2
36
What is asthma?
= long-term inflammatory disease of the airways, characterised by reversible airway obstruction and bronchospasm
37
Investigations to do in a child with asthma (5)
- detailed history
- serial peak flow readings, to indicate reversible airflow obstruction
- spirometry, to measure airflow capacity
- trial of a SABA
- FeNO testing where cases are unclear
38
What is an atrial septal defect (ASD)?
= cardiac malformation where a hole exists between the L and R atria. This is due to a defect in the septum secundum during cardiac embryonic development
39
Is atrial septal defect more common in females or males?
= females
40
Signs and symptoms of atrial septic defects? (3)
- often asymptomatic
- ejection sysolic murmur on auscultation (incidental finding), loudest on lower L sternal edge
- in decompensated patients: HF with SOB, fatigue, oedema, rapid HR
41
How are atrial septal defects diagnosed?
= echocardiogram
42
Management options for atrial septal defects? (2)
Depends on severity of lesion:
- most managed conservatively
- surgical closure for larger defects
(routine echocardiograms used to monitor ASDs for changes over time)
43
Complications associated with atrial septal defects? (2)
- heart failure
- paradoxical embolisms, can cause stroke
44
What is ADHD?
= attention deficit hyperactivity disorder (ADHD) is a neurodevelopmental disorder characterised by persistent patterns of inattention, impulsivity, and hyperactivity that are inappropriate for the individual’s developmental level
45
2 main symptoms in ADHD?
- inattention
- hyperactivity/ impulsivity
46
How long do symptoms need to be present for ADHD diagnosis according to DSM-5 criteria?
= 6 months
47
What is used to help diagnose ADHD?
= clinical criteria set by DSM-5
48
Conservative management options for ADHD? (2)
- behavioural techniques: CBT, behaviour therapy, psychoeducation, social skill training
- extra support at home
49
Does ADHD affect intellectual ability?
= no
50
Medical management of ADHD?
= stimulant medications (such as methylphenidate or amphetamines)
51
What do the medications used in ADHD do?
= have activity in frontal lobe, thus increasing executive function, attention, and reducing impulsivity
52
What are autistic spectrum disorders (ASD)?
= set of complex neurodevelopmental disorders, characterised by a spectrum of social, language, and behavioural deficits
53
Are males of females more likely to be diagnosed with an autism spectrum disorder?
= males (approx. 4:1)
54
Risk factors for autistic spectrum disorder? (3)
- advanced parental age at time of conception
- genetic mutations
- maternal exposure to specific drugs during pregnancy
55
What sort of SOCIAL interactions problems can children with an autism spectrum suffer from? (3)
- prefer to play alone
- avoid eye contact
- lack 'theory or mind' - don't think about others perspectives
56
What sort of LANGUAGE problems can children with an autism spectrum disorder suffer from? (4)
- speech + language delay
- monotomous tone of voice
- difficulty using pronours
- interpret speech literally
57
What sort of BEHAVIOURAL problems can children with an autism spectrum disorder suffer from? (3)
- narrow interests (e.g., trains)
- ritualistic behaviours that rely heavily on routine
- sterotypes movements (e.g., rocking, flapping hand movements)
58
What % of children with autism may also experience seizures?
= about 25%
59
Key strategies used in managing autism spectrum disorders? (2)
- behavioural management
- family support, due to chronic nature of disorder
60
Prognosis of autistic spectrum disorder: what % of children with ASD will be able to live independently as adults?
= less than 10%
61
What is bacterial tracheitis?
= severe, potentially fatal condition in the paediatric population
Characterised by high fever, and rapidly progressive airway obstruction due to accumulation of copious, thick airway secretions
62
Bacterial tracheitis vs croup?
= may present similarly but bacterial tracheitis tends to be more severe and rapidly progressive
Croup presents typically with a low grade fever compared to a high fever in bacterial tracheitis
63
Most common causative agent of bacterial tracheitis?
= Staphylococcus aureus
(usually follows a viral upper respiratory tract infection)
64
Management of bacterial tracheitis? (4)
- IV antibiotics (broad-spectrum until the causative organism is identified)
- intubation in severe cases
To help clear secretions:
- airway humidication
- chest physiotherapy
65
What is biliary atresia?
= rare but serious condition where the bile ducts in a new-born’s liver undergo progressive fibrosis + destruction
66
Signs and symptoms of biliary atresia? (2)
- prolonged jaundice (persisting beyond 14 days of life)
- dark urine and pale or chalky white stool (signs of biliary obstruction)
67
What type of bilirubin is raised in biliary atresia?
= conjugated bilirubin
(+ deranged LFTs)
68
Definitive diagnositc test for biliary atresia?
= cholangiography
(= imaging of bile duct by x-ray + an injection of contrast medium)
69
What is cholangiography?
= imaging of the bile duct by x-ray + injection of contrast medium
70
Management for biliary atresia?
= surgical intervention - hepatoprotoenterostomy
71
What is a hepatoprotoenterostomy? what is it used to treat?
= this surgery creates a new pathway from the liver to the gut to bypass the fibrosed bile ducts
Carried out to treat biliary atresia
72
A ependymoma is a brain tumour originating from the cells of the
- ventricular system
- pituitary gland
- ventricular system
73
A craniopahyngioma is a brain tumour originating from the cells of the
- ventricular system
- pituitary gland
- pituitary gland
74
Most common solid-organ malignancy?
= brain tumour
75
Inherited genetic conditions which may increase a child's risk of developign a brain tumour? (2)
- neurofibromatosis
- Li-Fraumeni syndrome
76
What is a branchial cyst?
= a congenital malformation that arises from an embryonic remnant. A painless, cystic mass located anterior to the sternocleidomastoid muscle just below the ear
77
Management options for a branchial cyst (conservative + active)
Conservative options: observation + antibiotics for infection
Active options: involves surgical excision of the cyst
78
What are reflex anoxic seizures (RAS)?
= paroxysmal, non-epileptic events seen in young children, which result from a temporary decrease in cerebral blood flow due to a reflex asystole or bradycardia
79
Are breath holding attacks and reflex anoxic seizures harmful?
= no, they are usually benign
80
Management of reflex anoxic seizures (RAS) and breath holding attacks? (2)
- reassurance about benign nature of these events
- avoid triggers
In very severe cases or frequent cases, referral to paediatric neurologist or cardiology may be necessary
81
How are breath holding attacks and reflex anoxic seizures diagnosed? (/2)
= primarily clinical
- can do ECG if cardiac cause suspected
82
What is bronchiolitis?
= = widespread chest infection, predominately affecting infants aged 1-12 months
This lower respiratory tract disease targets the bronchioles, causing inflammation and congestion
83
Most common cause of bronchiolitis?
= respiratory syncytial virus (RSV)
84
How it bronchiolitis diagnosed?
= primarily clinical diagnosis
(CXR can be consiered in severe cases)
85
What can be given as prophylaxis for bronchiolitis in high-risk patients?
= Palivizumab
86
Bronchiolitis: what can be used in severe cases?
= antiviral therapy (e.g., Ribavirin)
87
Complications of bronchiolitis?
= bronchiolitis obliterans aka, popcorn lung
88
What is bronchiolitis obliterans also known as?
= popcorn lung
89
What occurs in bronchiolitis obliterans (popcorn lung)?
= bronchioles are injured due to infection or inhalation of a harmful substance, leading to an overactive cellular repair process and subsequent build-up of scar tissue
The scar tissue obstructs the bronchioles, impairing oxygen absorption in the body. The scarring and narrowing of the bronchioles may continue to worsen over time, potentially leading to respiratory failure
90
What is bronchiolitis obliterans?
= pathological condition characterised by permanent obstruction of the bronchioles, the smallest airways in the lungs
This obstruction results from chronic inflammation that leads to the formation of scar tissue within these airways
91
Causes of bronchiolitis? (2)
- viral, most freuqnt Adenovirus
- complication following bone marrow or lung transplants
92
Most common viral cause of bronchiolitis obliterans?
= adenovirus
93
Investigations used in bronchiolitis obliterans? (4)
- CXR (can be normal)
- CT scan - detect early lung changes
- lung biopsy - confirm diagnosis
- pulmonary function tests
94
What is observed in FEV1 in patients with bronchiolitis obliterans?
= a significantly reduced FEV1 (16-21%) is often observed in bronchiolitis obliterans
95
Management of bronchiolitis obliterans? (2)
= primarily supportive
- can use immunosuppressive agents
96
What is caput seccedaneum?
= a neonatal condition that occurs as a result of the pressure exerted by the top of the infant’s skull against the dilating cervix during labour
It is characterised by a subcutaneous serosanguineous fluid collection superficial to the cranium but beneath the skin
97
Cause of caput seccedaneum?
= primary cause is sustained pressure from the dilating cervix, birth canal, or forceps on the infant’s head during labour
98
In caput seccedaneum - can the swelling cross cranial suture lines?
= yes
(unlike cephalohematoma)
99
Caput seccedaneum: imaging to help confirm diagnosis?
= USS
100
Management of caput seccedaneum?
= generally, self-resolving
Observe + reassure
101
How is caput seccedaneum diagnosed?
= primarily clinical
102
What is cerebral palsy?
= refers to a group of permanent movement disorders that appear in early childhood
This condition occurs due to damage to a child’s central nervous system (CNS), particularly areas involved in motor control. The lesions incurred is non-progressive
103
Which of the following categories of motor disorders associated with cerebral palsy is due to damage to pyramidal pathways?
- spastic
- dyskinetic/athetoid
- ataxic
- spastic
104
Which of the following categories of motor disorders associated with cerebral palsy is due to damage to the basal ganglia pathways?
- spastic
- dyskinetic/athetoid
- ataxic
- dyskinetic/athetoid
105
Which of the following categories of motor disorders associated with cerebral palsy is due to damage to cerebellar pathways?
- spastic
- dyskinetic/athetoid
- ataxic
- ataxic
106
Describe 'clasp-knife' spasticity?
= initial resistance when attempting passive movement, followed by rapid decrease in resistance
107
Imaging used in investigating suspected cerebral palsy?
= MRI - to visulise extent + nature of brain lesions
108
Medical options used in cerebral palsy (2)
- Baclofen, to manage muscle spasms
- Botox injection, to help with contractures
109
What is Baclofen used for in cerebral palsy?
= can be used to manage muscle spasms
110
What is chickenpox?
= = acute infectious disease caused by the varicella-zoster virus (VZV), a member of the herpes virus family
This highly contagious illness, predominantly seen in children, is characterised by a vesicular rash, mild fever and malaise
111
Cause of chickenpox?
= varicella-zoster virus (aka, human herpes virus 3 (HHV3))
112
Peak infectivity in chickenpox
(from when to when, days)
- 1-2 days before the rash appears until 5 days after the rash
113
How is chickenpox diagnosed?
= clinical, due to characteristic nature of rash and hx of exposure
114
What treatment is available for immunocompromised patients, previously unexposed pregnant women or neonate with peripartum exposure to chickenpox? (2)
- IV Aciclovir
- human varicella-zoster immunoglobulin (VZIG)
115
Management strategies/ advise for those suffering from chickenpox? (4)
- keep fingernails short to prevent iching + infection
- loose, long-sleeve clothing to limit skin exposure + scratching
- cooling measures like oatmeal baths or calamine lotion to reduce itching
- analgesics + antipyretics for symptom relief
116
What is coarctation of aorta?
= = congenital heart condition characterised by a narrowing of the aorta
This narrowing typically occurs just before the ductus arteriosus
117
Coarctation of the aorta is associated with which genitic condition?
= Turner Syndrome
118
Radio-femoral delay is suggestive of?
= coarctation of the aorta
119
How is coarctation of aorta diagnosed and monitored?
= echocardiography
120
Coarctation of aorta: what can be given to keep the ductus arteriosis patent until the defect can be corrected?
= prostaglandins
121
What is kernicterus?
= bilirubin encephalopathy, is bilirubin-induced neurological damage, which is most commonly seen in infants
It occurs when the unconjugated bilirubin (indirect bilirubin) levels cross 25 mg/dL in the blood from any event leading to decreased elimination and increased production of bilirubin
122
Cyanotic congenital heart disease (R>L)
(5T's with 1-5 mnemonic)
- Truncus arteriosus - vessels join to make 1
- Transposition of great vessels - 2 major vessels switched
- Tricuspid atresia - 3 (tricuspid)
- Tetralogy of Fallot - 4 defects
- Total anomalous pulmonary vascular return, 5 letters (TAPVR)
123
What cells produce surfactant?
= type II alveolar cells
124
What is lung compliance?
= a measure of the lungs ability to stretch and expand
125
At which week gestation fo the alveolar cells become mature enough the start producing surfactant? (range)
= between 24-34 weeks gestation
126
Which 2 hormones are released in response to stress of labour which help stimulate respiratory effort?
- adrenalin
- cortisol
127
What is the foramen ovale?
= hole between the L and R atria of the heart
128
What keeps the ductus arteriosus open?
= prostaglandins
129
How does a babys first breath cause the functional closure of the foramen ovale?
First breaths baby takes expand alveoli -> decreasing pulmonary vascular resistance -> this causes a fall in pressure in the R atrium -> at this point L atrial pressure greater R atrial pressure -> this squashes the atrial septum and causes functional closure of the foramen ovale -> this structurally closes and becomes fossa ovalis
130
What is the ductus arteriosus?
= a blood vessel that connects the pulmonary artery to the aorta
This conncection is present un all babies in the womb, but should close shortly after birth
131
Increased blood oxygenation causes
- a drop in prostaglandins
- an increase in prostaglandins
- a drop in prostaglandins
132
What does extended hypoxia to the brain in a neonate cause?
= hypoxia-ischaemic encephalopathy (HIE)
133
What can stimuate breathing in a neonate?
= vigorous drying
134
How are babies < 28 weeks kept warm?
= in a plastic bag, still wet, managed under a heat lamp
135
When is the APGAR score meant to be done? (3)
= 1, 5, and 10 minutes
136
In babies were there is suspected HIE (hypoxia-ischaemic encephalopathy), what may they benefit from?
= active cooling
137
Negative effect of delayed cord clamping?
= increase in neonatal jaundice
138
Benefits of delayed cord lamping (5)
- improved hb
- improved iron stores
- improved BP
- reduction in intraventricular haemorrhage
- reduction in necrotising enterocolitis
139
WHO recommends uncompromised neonates to have a delay in cord clamping of how long?
= at least 1 minute
140
What is given to babies as standard practice shortly after birth?
= vitamin K
(babies are born with deficiency of vitamin K)
141
Within how many days does the newborn exmaination need to be done?
= within 3 days (72 hours)
142
When is blood spot screening done? when is the latest it can be done?
= taken on day 5, day 8 at the latest
143
How many drops are required for the blood spot screening?
= 4 seperate drops
144
Which congenital conditions does the blood spot screening test for? (9)
- sickle cell anaemia
- cystic fibrosis
- congenital hypothyroidism
- phenylketonuria
- medium-chain-acyl-CoA
- dehydrogenase deficiency (MCAAD)
- maple syrup urine disease (MSUD)
- isovaleric acidaemia (IVA)
- glutaric aciduria type 1 (GA1)
- homocystin
145
3 questions to ask before doing newborn examination
- has the baby passed meconium?
- baby feeding okay?
- is there a family history of congenital heart, eye, or hip problems?
146
How might a duct-dependent congeintal heart condition be picked up in the newborn examination?
= difference in pre-ductal and post-ductal saturations
147
Newborn check: where can be pre-ductal and post-ductal saturations be measured?
Pre-ductal: measured in baby's R hand
Post-ductal: measured in either foot
148
Describe Barlow's + Ortolani manoeuvres
Barlow's manoeuvre = guiding the hips into mild adduction and applying a slight forward pressure with the thumb
Ortolani manoeuvre = hip is abducted and gentle pressure is applied to the proximal thigh from behind. Here, the examiner attempts to relocate an already dislocated femoral head back into the acetabulum. If the joint is dislocated, a palpable "clunk" is noticed as the head slides back into place
149
Newborn check: what is the moro reflex?
= when rapidly tipped backwards arms and legs will extend
150
Newborn check: what is the rooting reflex?
= tickling cheek will cause them to turn towards the stimulus
151
What is talipes?
= aka, clubfoot. Where the ankles are in a supinated position, rolled inwards
152
Positional vs structural talipes?
Positional talipes = where muscles are slightly tight around the ankle but the bones are unaffected. Foot can still be moved into normal position. Requires physiotherapy
Structural talipes = involves the bones of the foot and ankle, and requires referral to an orthopaedic surgeon
153
What is given to treat haemangiomas?
= beta-blocker (i.e., Propanolol)
153
Common causative organisms in neonatal sepsis (5)
- group B streptococcus
- e.coli
- listeria
- klebsiella
- staphylococcus aureus
154
First-line antibiotics in neonatal sepsis? (2)
= NICE recommends Benzylpenicillin + Gentamycin
155
What is hypoxic-ischaemic encephalopathy (HIE)?
= some hypoxia is normal during birth, however prolonged or severe hypoxia leads to ischaemic brain damage
HIE can lead to permanent damage to the brain, causing cerebral palsy. Severe HIE can result in death
156
What is Sarnat staging used to grade in neonates?
= hypoxic-ischaemic encephalopathy grades
157
Management of hypoxic-ischemic encephalopathy (2)
- supportive care
- therapeutic hypothermia
158
RBCs contain:
- conjugated bilirubin
- unconjugated bilirubin
- unconjugated bilirubin
159
When is jaundice classed as 'pathological' in a neonate?
= in the first 24 hours of life
160
What is haemolytic disease of the newborn?
= incompatibility between rhesus antigens on the surface of the RBCs of the mother and foetus. Causes haemolysis + jaundice in the neonate
161
What is classified as 'prolonged jaundice'? (2)
(in days, term + premature)
At term: > 14 days
In premature babies: > 21 days
162
Treatment of neonatal jaundice?
= phototherpay
163
What is apnoea of prematurity?
= defined as periods where breathing stops spontaneously for > 20 seconds, or shorter periods with oxygen desaturation or bradycardia
164
Management of apnoea of prematurity (2)
- tactile stimulation (apnoea monitor can beep when apnoea is occuring)
- IV caffeine - prevents apnoea + bradycardia
165
Describe how retinopathy of prematurity occurs? How is it associated with hypoxia?
= vessel formation is stimulated by hypoxia, which is a normal condition in the retina during pregnancy. When the retina is exposed to higher oxygen concentrations in a preterm baby, particularly with supplementary oxygen during medical care, the stimulant for normal blood vessel development is removed
When the hypoxic environment recurs, the retina responds by producing excessive blood vessels (neovascularisation) as well as scar tissue. These abnormal blood vessels regress and leave the retina without a blood supply. The scar tissue may cause retinal detachment
166
First-line management in retinopathy of prematurity?
= transpupillary laser photocoagulation, to halt and reverse neovascularisation
167
What is respiratory distress in a newborn usually from?
= premature neonates, born before the lungs start producing adequate surfactant
168
'Ground-glass' appearance on CXR in a newborn is suggestive of..?
= respiratory distress syndrome
169
What is necrotising enterocolitis?
= disorder affecting premature neonates, parts of the bowel become necrotic - life-threatening emergency
170
Risk factors for necrotising enterocolitis (5)
- low birth weight, premature
- formula feeds
- respiratory feeds
respiratory distress + assisted ventilation
- sepsis
- patent ductus arteriosis and other congenital heart disease
171
Imaging used to diagnose necrotising enterocolitis?
= abdominal x-ray
(supine position)
172
Management options in necrotising enterocolitis (5)
- nil by mouth
- IV fluids
- antibiotics
- total parenteral nutrition (TPN)
- surgical intervention, to remove dead bowel disease
173
What is neonatal abstinence syndrome?
= refers to withdrawal of symptoms that happens in neonates of mothers that used substances in pregnancy
174
True or false: SSRI withdrawal typically benefits from medical treatment
= false
175
What is coeliac disease?
= t cell-mediated inflammatory autoimmune disease that impacts the small bowel
Occurs when sensitivity to prolamin results in villous atrophy in the lining of the small intestine and malabsorption
176
What proteins are people with coeliac disease sensitive to?
= prolamin
177
Is coeliac disease more common in males or females?
= females
178
What is dermatitis herpetiformis associated with?
= coeliac disease - dermatological manifestation
179
Serological testing for coeliac disease (3)
- anti-TTG IgA antibodies
- IgA levels
- anti-TTG IgG (useful in those with IgA deficiency)
180
When might 'anti-TTG IgG' be useful in diagnosing coeliac disease?
= in those with an IgA deficiency
181
Gold-standard diagnostic test in those with suspected coeliac disease?
= oesophagogastroduodenoscopy (OGD) with duodenal/ jejunal biopsy
182
Presence of HLA-DQ2 allele is associated with what condition?
= coeliac disease
183
Mainstay management for coeliac disease?
= implementation of lifelong gluten-free diet + education
184
What is conduct disorder?
(age <)
= psychological diagnosis given to patients < 18 years of age who consistently exhibit behaviours and attitudes that disrespect and violate the rights of others, often breaching societal norms and rules
185
Is conduct disorder more common in males of females?
= males
186
Signs and symptoms of conduct disorder? (4)
- persistent + serious violation of rules
- physical aggression
- destructive behaviour
- decitfulness
187
Is prognosis of conduct disorder generally good or bad?
= generally poor
188
Management of conduct disorder (4)
- psychotherapy, CBT
- family therapy
- pharmacological treatment, used to manage comorbid conditions
- school-based interventions
189
190
Around what % of children with conduct disorder develop antisocial personality disorder or develop substance misuse issues?
= around 50%
191
What is congenital adrenal hyperplasia?
= represents a collection of autosomal recessive disorders characterised by impaired steroid hormone synthesis within the adrenal cortex due to enzyme defects
192
Most common type of congenital adrenal hyperplasia?
- deficiency in 18-hydroxylase
- deficiency in 21-hydroxylase
- deficiency in 30-hydroxylase
- deficiency in 21-hydroxylase
193
What diagnosis might the following symptoms suggest...
- ambiguous genitalia
- hypotension
- salt-wasting crises
- vomiting
- virilisation (=development of masculine secondary sexual characteristics)
= congenital adrenal hyperplasia
194
What occurs to the following hormones in a child with congenital adrenal hyperplasia?
- 17-hydroxyprogesterone
- ACTH
- crotisol
- 17-hydroxyprogesterone, elevated
- ACTH, elevated
- crotisol, low
195
Acute treatment of congenital adrenal hyperplasia? (2)
- IV fluids
- hydrocortisone (for its glucocorticoid + mineralocorticoid effects)
196
What 2 medications are usually given to provide hormonal replacement in those with congenital adrenal hyperplasia?
- hydrocortisone
- fludrocortisone
(as needed)
197
What can be seen on ECG in congenital heart block?
= complete dissociation between p waves (representing atrial contraction), and QRS complexes (representing ventricular contraction)
198
What is congenital heart block associated with? (maternal/ 2)
- SLE
- anti-Ro and anti-La (sjogren's syndrome)
199
Signs and symptoms seen in congential heart block (2)
Neonates: bradycardia, or circulatory shock
Older children: pre-/ syncope
200
Primary investigation in congenital heart block?
= ECG
201
Management of congential heart block? (2)
If asymptomatic = close monitoring
Symptomatic = implantation of pacemaker
202
What is congenital hypothryroidism?
= paediatric endocrine disorder characterised by insufficient production of thyroid hormones at birth, leading to potential physical and neurodevelopmental complications if not promptly treated
203
Primary vs secondary congential hypothyroidism
Primary: thyroid dysgensis ( defect in thyroid gland development), or dyshormonogenesis (defect of thyroid hormone biosynthesis) - incldes thyroid agenesis, ectopic thyroid tissue and hypoplastic thyroid gland
Secondary: defects in hypothalamus or pituitary gland, leading to low TSH secretion
204
Neonate presents with large anterior fontanel, an umbilical hernia and distended abdomen, macroglossia (=large tongue) and weight gain.
What is the diagnosis?
= congenital hypothyroidism
205
206
207
208
Confirmatory tests for investigating congenital hypothyroidism (TSH + T4)
TSH: elevated
free T4: low
209
Scan to investigate congenital hypothyroidism (2)
- thyroid USS
- radionuclide scan (to identidy thyroid dysgenesis)
210
Management of congenital hypothryroidism? (2)
- Levothyroxine (thyroid hormone replacement therapy)
- regular monitoring for dose adjustments (TSH + T4 levels)
211
Paediatrics: what is constipation defined as?
= clinical condition wherein the child defaecates fewer than 3 times per week, or experiences significant difficulty passing stool
212
Most common cause of chronic constipation in children?
= dietary factors
(low fibre, avoidance using toilet, pain upon passing due to anal fissure, unrecognised sensation of needing to pass stool)
213
How is constipation in a child investigated?
- history
- palpation of impacted faeces (hard, depressible masses) on abdominal examination
214
Initial treatment for constipation in a child?
= Movicol disimpaction regimen
215
What is Hirschsprung's disease?
= a birth defect in which some nerve cells are missing in the large intestine, so a child’s intestine can’t move stool and becomes blocked
216
How is Hirschsprung's disease diagnosis confirmed?
= rectal suction biopsy (= removal of tiny pieves of tissue from the rectal area)
217
Definitive management in Hirschsprung's disease?
= surgical removal of the section of the aganglionic colon
The healthy bowel is then pulled out
218
What is laryngotracheobronchitis also known as?
= croup
219
What is croup?
= an acute respiratory syndrome which affects the larynx, trachea, and bronchi
It is characterised by inflammation and swelling that results in partial obstruction of the upper airway
220
Primary aetiological agent in croup?
= parainfluenza virus
221
Barking or seal-like cough in a child < 3 is suggestive of?
= croup
222
X-ray of the neck shows classic 'steeple sign' - what is this suggestive of?
= croup, indicative of subglottic narrowing in severe or atypical cases
223
What can be given in mild cases of croup in primary care?
= single dose of Dexamethasone
224
If child is admitted to hospital for croup, what treatment is usually given? (3)
- monitoring
- ocygen supplementation
- nebulised budesonide (=corticosteroid)
225
In a child with croup, if there are high concerns about airway patency what can be given?
= nebulised adrenaline
226
Example of congenital cyanotic heart defects (3)
- transposition of the great arteries
- pulmonary + tricuspid atresias
- tetralogy of fallot
227
What is cyanotic heart disease?
= encompasses a range of congenital heart defects resulting in a R to L shunt, which leads to systemic arterial desaturation and subsequent cyanosis
228
What might suggest cyanotic heart disease in a newborn?
= appears cyanotic
229
How is cyanotic heart disease diagnosed? (2)
- often antenatally during routine USS scans
- echocardiography - used to characterise the anatomy and heart function
230
Definitive management options in cyanotic heart disease?
- surgical correction
- if not correctable, heart transplant may be considered
231
What can be given in a child with cyanotic heart disease whilst awaiting definitive correction?
= prostaglandin E
(to maintain patency of the ductus arterioles, which can provide temporary relief from cyanosis)
232
Cystic fibrosis is...
- autosomal dominant
- autosomal recessive
- autosomal recessive
233
What is cystic fibrosis?
= progressive, autosomal recessive disorder that causes persistent lung infections and limits the ability to breathe over time
234
Mutation in the CFTR protein is associated with which condition?
= cystic fibrosis
235
Most common mutation associated with cystic fibrosis?
= Delta-F508
236
A sign of cystic fibrosis in neonate?
= meconium ileus, due to viscous meconium that causes a delay in passing and possible GI obstruction
237
Defintive diagnosis test for cystic fibrosis?
= sweat test
238
Cystic fibrosis: What does the sweat test measure?
= measures concentration of chloride in excreted sweat
239
Prophylactic medications taken in cystic fibrosis (3)
- antibiotics
- bronchodilators
- medicaitons to thin secreations
240
What is dornase alfa used for, what does it do?
= used in cystic fibrosis, thins secretions
241
Which regular immunisations are recommended for those with cystic fibrosis? (2)
- penumococcal vaccines
- influenza vaccines
242
What is delayed puberty defined as? (2)
Absence of any signs of pubertal development by the age of
- 14 in boys
- 13 in girls
243
Most common cause of delayed puberty?
= constutitional delay of growth and puberty
244
How is constitutional delay of growth and puberty confirmed?
= hand-wrist- x-ray to assess bone age
(delated in contitutional bone delay due to late fusion of the epiphyseal plates)
245
Investigations in delayed puberty (3)
- hand-wrist x-ray
- endocrine function tests
- genetic testing
246
Management in delayed puberty (2)
- observational, with reassurance that puberty will eventually occur
- hormone therapy might be considered
247
The following features are associated with which condition?
- lack of breast development
- shield chest
- widely spaces nipples
- webbed kneck
- cubital valgus
- coarctation of the aorta
= Turners syndrome (XO)
248
249
What is developmental dysplasia of the hip (DDH)?
= congenital abnormality of the hip joint in which the ball of the femur (femoral head) and the socket of the pelvis (acetabulum) do not articulate approximately
This malalignment can result in the joint dislocating easily and continuing to develop abnormally
250
Development dysplasia of the hip (DDH): Risk factors, 5 Fs?
- female
- firstborn
- family history
- frank breech presentation
- fluid, oligohydramnios
251
252
253
254
255
Which of the following tests for developmental dysplasia of the hip, tests for posterior dislocation?
- Barlows
- Ortolani
- Barlows
(Barlow, take that)
255
Which of the following tests for developmental dysplasia of the hip, tests for relocation on hip abduction?
- Barlows
- Ortolani
- Ortolani
256
Imaging used to help confirm developmental dysplasia of the hip?
= hip ultrasonography
256
What is a pavlik harness used for?
= keeps hips in a flexed and abducted position, used in developmental dysplasia of the hip (DDH)
257
What is Downs syndrome?
= Trisomy 21, is a genetic condition resulting from the presence of 3 copies of chromosome 21, rather than the typical 2
258
Facial features associated with Downs syndrome (7)
- upward-slanting palpebral fissures
- epicanthic folds
- protruding tongue
- small low-set ears with adherent earlobes
- hypoplastic nasal bridge
- broad, flat + small oral cavity
- single transverse palmar crease
259
What conditions is Downs syndrome associated with?
- learning difficulties
- congenital heart defects
260
What is eidenmenger syndrome?
= = pathological medical condition where in a congenital L to R heart shunt reverses into a R to L shunt
This reversal is typically secondary to pulmonary HTN and is associated with R ventricular hypertrophy
261
What causes Eisenmenger syndrome?
= increased pulmonary pressures
262
Signs and symptoms of Eidenmenger syndrome
- usually presents in late teens
- main feature is cyanosis
263
Gold standard investigation for diagnosis of Eisenmenger
= cardiac catheterization
264
Management for patient with Eisenmenger syndrome (2)
- heart-lung transplant
If not feasible: palliative care
265
What is duodenal atresia?
= congenital malformation characterised by the presence of a blind-ending duodenum, which is not patent
This abnormality leads to an obstruction of the GI tract, with resulting clinical implications
266
What condition is duodenal atresia associated with?
= down's syndrome
267
Antenatal signs of duodenal atresia?
= polyhydraminos
(due to inadequate ingestion of amniotic fluid by foetus)
268
Postnatal sign of duodenal atresia? (2)
- distended abdomen
- vomiting
269
What does the 'double bubble' sign on abdominal x-ray suggest?
= duodenal atresia
(1 gas bubble visible in the stomach, and another in the proximal, patent part of the duodenum prior to the atresia)
270
Primary management option for patient with duodenal atresia?
= surgical repair - duodenoduodenostomy
(procedure involves reconnecting the closed proximal and distal segments of the duodenum to alleviate the obstruction)
271
What is DKA characterised by? (3)
- hyperglycaemia
- acidosis
- ketonaemia
272
Is a high temperature usually a symptoms of DKA?
= no - could indicate an underlying infection that has triggered the DKA
273
DKA: If patient is alert, and not significantly dehydrated, how would you manage? (2)
(assume can swallow)
- encourage oral fluid intake
- aminister SC insulin
274
DKA: If pateint vomiting, confused, or significantly dehydrated, how would you manage? (2)
- IV fluids (initial bolus of 10ml/kg 0.9% NaCl, then discuss with senior) - if evidence of shock, initial bolus should be 20ml/kg
- insulin infusion (at 0.1 units/kg/hour 1 hour after starting IV fluids)
275
Major complications of DKA management?
= cerebral oedema
276
How are enteroviruses usually spread? (2)
- faecal-oral route OR,
- droplet transmission
277
Enteroviruses: Which of the following commonly causes hand foot-and-mouth disease?
- coxsackie A virus
- coxsackie B virus
- poliovirus
- echoviruses
- coxsackie A virus
278
Enteroviruses: Which of the following commonly causes myocarditis?
- coxsackie A virus
- coxsackie B virus
- poliovirus
- echoviruses
- coxsackie B virus
279
Enteroviruses: Which of the following commonly causes poliomyelitis?
- coxsackie A virus
- coxsackie B virus
- poliovirus
- echoviruses
- poliovirus
280
Enteroviruses: Which of the following commonly causes aseptic meningitis?
- coxsackie A virus
- coxsackie B virus
- poliovirus
- echoviruses
- echoviruses
281
Key investigations for diagnosing enteroviral infections (3)
- stool cultures
- throat cultures
- CSF analysis (in cases of suspected CNS involvement)
282
How are enteroviral infections typically managed?
= supportive treatment
(analgesia, hydration, antipyretics, ventilatory support, vaccination against polio)
283
What is Ewing's sarcoma?
= malignant, small round cell tumour that primarily involves the bone but can also arise in soft tissues
284
Second most prevalent bone cancer in children + adolescents?
= Ewing's sarcoma
285
Defintive diagnosis for Ewing's sarcoma is established how?
= bone biopsy, which is examined histologically and genetically
286
How is Ewing's sarcoma managed? (3)
- surgery, to remove primary tumour + metastatic disease
- chemotherapy, reduce tumour size
- radiotherapy, used as adjuvant therapy or when surgery is not feasible
287
What are innocent murmurs in children?
= often referred to as functional or physiological murmurs, are benign heart sounds that can be detected in a significant proportion of healthy children
288
How common are innocent murmurs in children?
= very common, up to 1/3 of children will have one at some point during their childhood
289
6 S's - features of innocent heart murmurs in a child?
- Soft, faint not loud
- Systolic (NOTE: all diastolic murmurs are pathological)
- Sensitive – murmur’s intensity changes with child’s position or alongside respiration
- Short: not holosystolic (does not last for the entire systolic phase)
- Single: no additional sounds accompanying the murmur
- Small: murmur localised and does not radiate to other areas
290
True or false: soft, diastolic murmur with no other added sounds is suggestive of an innocent heart murmur in a child?
= false, all diastolic murmurs are pathological
291
Innocent heart murmur in a child: If the murmur persists, or does not have typical features of an innocent murmur, what further investigations may be done? (3)
- ECG
- echocardiogram
- CXR
292
What is a febrile convulsion?
= = type of seizure that occur in association with a fever, without evidence of intracranial infection, or defined cause
These seizures are typically short-lived, lasting < 15 minutes, and are tonic-clonic in nature
293
Features of a simple febrile convulsion (3)
- short-lived, < 15 minutes
- tonic-clonic in nature
- associated with fever
294
Features of a complex febrile convulsion (3)
- partial or focal seizures
- last > 15 minutes
- occur multiple times during same febrile illness
295
What are the chances of a child having another febrile convulsion after the first one?
= 1/3
296
Are febrile convulsions known to cause any lasting damage?
= no
297
What are the risks of developing epilepsy
- for general population
- after simple febrile convulsion
- after complex febrile convulsion
General population: 1.8%
Simple febrile convulsion: 2-7.5%
Complex febrile convulsion: 10-20%
298
What is foetal alcohol syndrome (FAS)?
= represents a set of physical and mental birth defects associated with prenatal exposure to alcohol
299
Facial features associated with foetal alcohol syndrome (FAS) (3)
- short palpebral fissures (= small eyes, small eyelid width)
- smooth philtrum (= groove under nose + above lip)
- thin upper lip
300
Can you cure foetal alcohol syndrome (FAS)?
= no, supportive management
301
What is fragile X syndrome?
= genetic disorder that leads to a range of developmental problems including learning disabilities and cognitive impairment
It is caused by a mutation in the FMR1 gene, which usually makes a protein needed for brain development.
302
Mutation of the FMR1 gene results in the development of which condition?
= fragile X syndrome
(FMR1gene located on x-chromosome - CGG trinucleotide repeat expansion)
303
- long face
- protruding ears
- intellectual impairment
- large testes
- social anxiety
- autistic spectrum features
Are all associated with what genetic condition?
= fragile x-syndrome
304
What is the most common inherited form of learning disabilities?
= fragile x syndrome
305
What are fraser guidelines used for?
= set of criteria that must be met for a health professional to give advice to a child < 16 regarding contraception and sexual health without breaking confidentiality
306
Fraser guidelines (5)
- can understand nature of implications of proposed treatment
- cannot be persuaded to tell their parents
- likely to continue having sexual intercourse
- physical or mental health likely to suffer
- in person's best interest
[if all of the above criteria are met, the child is deemed to be ‘Fraser competent’ and can therefore receive advice without breaking confidentiality]
307
Why is GORD commonly seen in babies?
= due to immaturity of the lower oesophageal sphincter, allowing contents to pass freely into the oesophagus from the stomach
308
First-line management of GORD in children
= reassurance + practical advice
- small frequent meals
- burping regularly
- not over-feeding
- keep baby upright
309
What is gastroenteritis?
= inflammation of the GI tract, predominantly involving the stomach + small intestines, characterised by diarrhoea and vomiting
310
Most common cause of viral gastroenteritis in infants?
- adenovirus
- norovirus
- rotavirus
- rotavirus
311
Most common cause of viral gastroenteritis across all ages?
- adenovirus
- norovirus
- rotavirus
- norovirus
312
Gastroenteritis: Which of the following is found in cooked meats + cream products?
- staphylococcus aureus
- bacillus cereus
- clostridium perfringens
- campylobacter
- e. coli
- staphylococcus aureus
313
Gastroenteritis: Which of the following is associated with reheated rice?
- staphylococcus aureus
- bacillus cereus
- clostridium perfringens
- campylobacter
- e. coli
- bacillus cereus
314
Gastroenteritis: Which of the following is commonly found in reheated meat dishes or cooked meats?
- staphylococcus aureus
- bacillus cereus
- clostridium perfringens
- campylobacter
- e. coli
- clostridium perfringens
315
Investigations for gastroenteritis (2)
- usually clinical
- may include stool cultures + microscopy
316
Management of gastroenteritis (2)
= primarily supportive
- fluid replacement
- antibiotics (if indicated - systemically unwell, immunosuppressed, elderly)
317
Gastroenteritis: Which of the following antibiotics is used against Salmonella + Shigella?
- Ciprofloxacin
- Amoxicilin
- Erythromycin
- Tetrocycline
- Ciprofloxacin
318
Gastroenteritis: Which of the following antibiotics is used against Campylobacter?
- Ciprofloxacin
- Amoxicilin
- Erythromycin
- Tetrocycline
- Erythromycin (macrolide)
319
Gastroenteritis: Which of the following antibiotics is used against Cholera?
- Ciprofloxacin
- Amoxicilin
- Erythromycin
- Tetrocycline
- Tetrocycline (used to reduce transmission)
320
Is food poisoning a notifiable disease in the UK?
= yes
321
Gastroenteritis: How to prevent norovirus outbreak?
= handwahsing with soap + warm water
322
What is glandular fever?
= viral infection primarily caused by the Epstein Barr virus
This infectious disease is characterised by symptoms such as
- Fever
- Fatigue
- Sore throat
- Possible hepatomegaly or splenomegaly
323
What is glandular fever also known as? (2)
- infectious mononucleosis
- 'kissing disease'
324
Primary cause of glandular fever (infetious mononucleosis)?
= Epstein Barr virus
325
How is gladular fever transmitted?
= saliva, which is why it is referred to as the ‘kissing disease’
However, virus can be spread through items such as eating utensils, and toothbrushes
326
What does a positive heterophile antibody 'Paul Bunnell' test suggest?
= glandular fever/ infectious mononucleosis
327
Why are patients with glandular fever recommended to avoid contact sport + heavy lifting for a minimum of 1 month?
= to minimise the risk of splenic rupture
328
Management of glandular fever?
= supportive - rest, OTC medicaiton for fever + sore throat
329
What is henoch schonlein purpura (HSP)/ IgA vasculitis ?
= is an immune-mediated small vessel vasculitis
It predominately affects children, especially those aged 3-5 years
330
Most common vasculitis in children?
= henoch schonlein purpura/ IgA vasculitis
331
Which vasculitis is associated with a Hx of a recent upper respiratory tract infection?
= henoch schonlein purpura/ IgA vasculitis
332
3 year old patient presents with purpura on lower limbs, abdominal pain, arthritis, + haematuria, 2 weeks ago he suffered from an upper respiratory tract infection.
What is the diagnosis?
= henoch schonlein purpura/ IgA vasculitis
333
Is henoch schonlein purpura more common in males or females?
= slightly more common in males
334
Following an episodes of henoch schonlein purpura, how long should the patient have regular urine tests to monitor for potential renal impairment?
= 12 months
335
What are the chances of recurrence of HSP?
= seen in 1/3 pateints
336
What is hodgkin's lymphoma?
= malignant lymphoma characterised by presence of Reed-Sternberg cells
337
B symptoms
- fever
- weight loss
- night sweats
338
4 types of Hodgkin's lymphoma
- lymphocyte predominant
- nodular sclerosis
- mixed picture
- lymphocyte deplated
339
What was 'ann arbor staging system' used to stage?
= Hodgkin's lymphoma
340
The presence of which cells on blood film is suggestive of Hodgkin's lymphoma?
= Reed-Sternberg cells
341
Scans which can be used to stage Hodgkin's lymphoma (2)
- CT scan
- PET scan
342
What does a high LDH on bloods suggest?
= indicates organ or tissue damage
343
How is Hodgkin's lymphoma treated?
= chemoradiotherapy
344
Hodgkin's lymphoma: Which of the following type is associated with a better prognosis?
- lymphocyte-predominant
- lymphcyte-deplete
- lymphocyte-predominant
345
What is a hydrocele?
= = pathological accumulation of serous fluid in a sac-like cavity specifically around the testicle
Typically presents as an enlarged scrotum
346
Primary hydrocele vs secondary hydrocele?
PRIMARY HYDROCELE = the processus vaginalis should typically obliterate after the descent. If it doesn't close completely, fluid from the abdomen can gradually accumulate in the scrotum, causing a hydrocele
SECONDARY HYDROCELE = can develop at any time, occur when there is excessive fluid produced within tunica vaginalis
347
Primary diagnostic tool in a suspected hydrocele?
= USS
348
Management options for a hydrocele (2)
- observation, many resolve by 12 months of age
- if persists 2 years, or causes discomfort - surgical correction
349
What is hydrocephalus?
= neurological disorder caused by the excessive accumulation of the CSF within the ventricular system of the brain
350
2 types of hydrocephalus
- communicating hydrocephalus
- non-communicating hydrocephalus
351
What does the following signs in a child suggest
- enlarged head
- bulging anterior fontanelle
- distension of scalp veins
- 'sunsetting' of the eyes
= hydrocephalus
352
How do you investigate a child with susepcted hydrocephalus?
- primarily imaging, cranial USS firstly
- MRI or CT brain for more detailed images
353
What is a ventricularoperitoneal shunt used for? in which condition?
= commonly inserted to divert excess CSF from brain
Used in the treatment of hydrocephalus
354
What is hypoxic ischaemic encephalopathy?
= condition characterised by brain damage resulting from antenatal or perinatal hypoxia
355
What is hypoxic ischaemic encephalopathy caused by?
= lack of oxygen in foetal circulation > leads to insufficient oxygen supply to the brain
356
Imaging used to assess extent of brain injury in hypoxic ischaemic encephalopathy
= mutiple MRI brain scans
357
What can be used to prevent further brain damage from secondary reperfusion injury in hypoxic ischaemic encephalitis?
= cooling therapy
358
What is immune thrombocytopenic purpura (ITP)?
= autoimmune condition, characterised by a reduction in the number of circulating platelets
It is a type II hypersensitivity reaction whereby the spleen produced antibodies are directed against the glycoprotein IIb/ IIIa or Ib-V-IX complex
359
What type of hypersensitivity reaction is immune thrombocytopenic purpura (ITP)?
= type II hypersensitivity reaction
360
ITP: In children, often presents as:
- self-limiting disease following a viral infection
- chronic disease, with relapsing course
- self-limiting disease following a viral infection
361
362
363
ITP: In adults, often presents as:
- self-limiting disease following a viral infection
- chronic disease, with relapsing course
- chronic disease, with relapsing course
364
Immune thrombocytopenic purpura (ITP): How is it managed?
= generally conservative, watch-and-wait approach
(high risk of spontaneous remission)
365
Immune thrombocytopenic purpura (ITP): Are platelet transfusions recommended?
= no, can increase rate of platelet destruction
366
Immune thrombocytopenic purpura (ITP): pharmacological management options for persistent cases (2)
- steroids
- IVIG (IV immune globulin)
367
Immune thrombocytopenic purpura (ITP): what may be considered in refractory cases?
= spelenctomy
368
What is impetigo?
= highly contagious superficial epidermal infection of the skin
Primarily caused by Staphyloccus and Streptococcal bacteria
369
Main causative agents in impetigo (2)
= mainly bacterial
- Staphylococcus aureus
- Group A Streptococcus
370
What skin condition presents with a charactersitic golden crust and is highly infectious?
= impetigo
371
Patient with impetigo has been advised to start Abx however, is allergic to penicillin, which of the following can be used instead?
- Flucloxacillin
- Clarithromycin
- Erythromycin
- Clarithromycin
372
Patient with impetigo has been advised to start Abx however, is pregnant, which of the following can be used instead?
- Flucloxacillin
- Clarithromycin
- Erythromycin
- Erythromycin
373
What cream can be used to treat localised non-bullous impetigo?
= hydrogen peroxide 1% cream
(apply 2 or 3 times daily for 5 days)
374
What is intussusception?
= refers to the invagination (telescoping) of a segment of the proximal bowel into a distal bowel segment
375
Child presents with being off his feeds, abdominal distention and 'recurrent jelly' stool.
What is the likely diagnosis?
= intussusception
376
Primary investigation in intussusception?
= abdominal USS
377
What does the 'target sign' on USS suggest?
= intussusception
378
Initial management of intussusception (if child is stable vs. not) (2,1)
- rectal air insufflation
- contrast enema
If child not stable:
- operative reduction
379
What is juvenile dermatomyositis (JDM)?
= paediatric variant of dermatomyositis, a systemic autoimmune disease primarily affecting the skin and muscles
380
What can be given for acute flate of juvenile dermatomysositis?
= steroids
381
What is juvenile idiopathic arthritis (JIA)?
= collective term used to describe a group of arthritis’s that affect children and young individuals < 16, where the aetiology is unknown. It is a diagnosis of exclusion characterised by persistent joint swelling that lasts > 6 weeks
382
How is juvenile idiopathic arthritis managed? (4)
Emotional + psychological support crucial - as extremelly debilitating
Pharmacological:
- NSAIDs - symptoms control
- Steroids - inflammation
- steroid-sparing agents (Methotrexate or TNF-alpha inhibitor) may be used to minimise long-term steroid use
383
What is kawasaki disease?
= medium-vessel vasculitis predominately affecting children
384
Kawasaki disease diagnostic criteria? (1,5)
Based on presence of high-grade fever persisting > 5 days
Accompanied by 4/5 of the following features ('CREAM')
C - conjuncitivits
R - rash (any non-bullous rash)
E - oedema/ erythema of hands + feet
A - adenopathy/ lymphadenopathy (usually unilateral + non-tender)
M - mucosal involvement (strawbery tongue, oral fissures)
385
Primary investigation in kawasaki disease?
= echocardiogram, due to risk of coronary aneurysm
386
Complication associated with kawasaki disease
= coronary aneurysms
387
Management of kawasaki disease (3)
- intravenous immunoglobulins (IVIg)
- high-dose aspirin
Regular echocardiograms
Close monitoring as recovery can take several weeks
388
Long QT-syndrome (LQTS) can increase the chances of what complications in young people?
= sudden cardiac death
(increased risk of ventricular fibrilation + cardiac arrest)
389
Main manifestation of long QT-syndrome?
= syncope, usually following exertion, emotional stress or during sleep
390
What is the cut-off for a pathological QT interval?
= interval > 480ms is considered pathological
391
What may be used in patients with a high risk, with long QT-syndrome?
= implanatable defibrillator
392
What is malrotation?
= congenital anomaly in which the midgut undergoes abnormal rotation and fixation during embryogenesis
The misplacement of the gut makes it susceptible to volvulus
393
Common complication of malrotation?
= volvulus
394
How might a neonate with malrotation present?
= bilious vomiting occuring within the first day of life
395
Gold-standard investigation for diagnosing malrotation?
= upper GI contrast study
396
How is malrotation managed?
= urgent surgical intervention to relieve obstruction, and correct anatomical abnormality
397
What is measles?
= highly contagious disease caused by the measles morbillivirus. It is transmitted via droplets from the nose, mouth, or throat of infected persons
398
What are 'koplik spots', and what are they pathognomonia for?
= small grey discolorations of the mucosal membranes in the mouth
Pathognomonic for measles
399
First line investigation for measles?
= measles-specific IgM and IgG serology (ELISA)
Most sensitive 2-14 days after onset of the rash
400
Management of measles
= mostly supportive care
401
What is important to give children < 2 with measles?
= vitamin A
402
Complications of measles (3)
- acute otitis media
- bronchopneumonia
- encephalitis
403
What is meckel's diverticulum?
= small outpouching extending from the wall of the intestine and located in the lower portion of the asmall intestine
404
What is the 'rule of 2s', in relation to Meckel's diverticulum? (4)
- 2:1 (M:F)
- 2 inches long
- 2 feet proximal to caecum
- affects 2% of population
405
What scan is used to investigate a suspected Meckel's diverticulum?
= CT scan
406
Meckle's diverticulum: What is a 99mTC scan used to identify?
= ectopic gastric mucosa
407
Management of Meckle's diverticulum?
= surugical resection (either wedge excision or small bowel resection and anastomosis)
408
Complciations of Meckle's diverticulum? (3)
- intussusception
- obstruction
- ulceration and perforation
409
What is meconium aspiration syndrome (MAS)?
= triggered by the passage of meconium from the amniotic fluid into the foetal lungs, leading to blockage and inflammation of the airways
410
What is meconium ileus?
= condition characterised by the thickening of meconium causing obstruction in the neonatal bowel
411
Meconium ileus can be an early indicator of what condition?
= cystic fibrosis
412
What % of meconium ileus cases are linked to cystic fibrosis?
- 30%
- 50%
- 90%
- 90%
413
Investigation for suspected meconium ileus?
= abdominal x-ray
414
Abdominal x-ray: ‘bubbly’ appearance of the intestines without air-fluid levels is indicative of what condition?
= meconium ileus
415
Meconium ileus: What does 'drip and suck' entail? (2)
- IV fluids
- stomach decompression w/ Ryles tube
416
What is meningitis?
= an inflammatory condition involving the meninges, which are the protective membranes covering the brain and spinal cord
417
Patient presents with fever, severe headache, neck stiffness, photophobia and confusion, what is the likely diagnosis?
= meningitis
418
Investigations for meningitis (3)
- lumbar puncture + CSF analysis
- blood cultures
- neuroimaging (CT or MRI)
419
Management of meningitis? (4)
- broad-spectrum antmicrobias
- antivirals (if viral cause)
- corticosteroids, reduce inflammation
- supportive management
420
What is a meningococcal infection?
= disease caused by Neisseria meningitidis bacterium, a gram-negative intracellular diplococcus
It resides in the nasopharynx of many children + young adults, with some strains having the potential to invade the bloodstream and cause fatal septicaemia
421
What is waterhouse-friedrichsen syndrome? and what is it associated with?
= rare, but life threatening disorder assocaited with bilateral adrenal haemorrhage
Associated with meningococcal infection
422
How is meningococcal infection investigated? (2)
- cultures, blood and CSF
- PCR testing for Neisseria meningitidis (highly sensitive)
423
Causative agent in meningococcal infection?
= Neisseria meningitidis
424
Is meningococcal infection a notifiable disease?
= yes
425
What is mesenteric adenitis?
= reactive inflammatory condition that primarily involves the mesenteric lymph nodes in the abdomen
Usually occurs after initial infection has resolves
Is a common mimic of appendicitis
426
Mesenteric adenitis is
- active inflammation of the lymph nodes
- a reactive process which usually occurs after initial infection has resolved
- a reactive process which usually occurs after initial infection has resolved
427
What is mesenteric adenitis commonly confused for?
= appendicitis
428
What imaging can help diagnose mesenteric adenitis?
= USS abdomen
(revelas enlarged mesenteric lymph nodes and a normal appendix)
429
Management of mesenteric adenitis?
= self-limiting, observe and reassure
430
What is minimal change disease?
= condition that primarily affects the kidneys. Despite causing significant clinical symptoms, the disease is marked by minimal or no changes visible under light microscopy in nephrological structures
(however, more subtle changes can be detected using electron microscopy)
431
Most common cause of nephrotic syndrome in children?
= minimal change disease
432
5 year old child presents with facial swelling, fatigue, and has noticed his urine is more fronthy then usual. He had a viral upper viral respiratory infection 2 weeks ago.
What is the likely diagnosis?
= minimal change disease
(most common cause of nephrotic syndrome in a child)
433
First-line pharacological treatment in minimial change disease?
- corticosteroid therapy, Prednisolone
+ fluid restriction
434
What can be given in severe cases of minimal change disease with significant fluid overload? (2)
- human albumin
- furosemide
435
Prognosis of minimal change disease (1/3,1/3,1/3)
1/3: resolve completely
1/3: relapses requiring additional steroid treatment
1/3: dependent on continued steroid/ immunosuppression therapy
436
What is muscular dystrophy?
= refers to a group of inherited genetic disorders characterised by the progressive degeneration and weakening of the body’s muscles
437
2 most common types of muscular dystrophy?
- Duchenne's muscular dystrophy
- Becker muscular dystrophy
438
Muscular dystrophy is
- autosomal dominant
- automomal recessive
- x-linked recessive disorder
- x-linked dominant disorder
- x-linked recessive disorder
(predominantly males affected)
439
Which protein is affected in muscular dystrophy?
= dystrophin gene
440
Which of the following types of muscular dystrophy presents in early childhood?
- Duchenne's MD
- Becker's MD
- Duchenne's MD
441
Which of the following types of muscular dystrophy is associated with dystrophin gene expressed at lower levels, and dysfunctional?
- Duchenne's MD
- Becker's MD
- Becker's MD
(in DMD, protein is virtually absent)
442
What is a positive Gower's manoeuvre a sign of?
= duchenne's muscular dystrophy
443
Describe a positive Gower's manoeuvre
- gets into prone position
- forms a triangle
- climbs on own thighs to stand up
(due to proximal muscule weakness)
444
Gold-standard test for diagnosing muscular dystrophy?
= genetic testing
445
Apart from genetic test, what can be used as a first-line screening tool for muscular dystrophy?
= creatinine kinase (CK)
Levels significantly high
446
Muscular dystrophy: what medical management can be used to help slow muscle degeneration?
= glucocorticoids
447
What is nocturnal enuresis also known as?
= bedwetting
448
What is nocturnal enuresis?
= also referred to as bedwetting, condition in which an individual, usually a child, involuntarily urinates during sleep
considered to be a typical part of development until the age of 5
449
Up to what age is nocturnal enuresis typical of normal development?
= up to the age of 5
450
Primary vs secondary nocturnal enuresis?
Primary: involves children who have never achieved urinary continence overnight
Secondary: refers to children who have previously achieved night-time continence but have subsequently lost it
451
Investigations for primary nocturnal enuresis? (3)
- history
- examination
- urine dip
452
1st line management option for nocturnal enuresis?
= nocturesis alarm
453
What is a nocturesis alarm, what does it do?
= device that detects moisture in underwear, activating ana alarm and alerting child to wake up and go to the bathroom
These alarms are generally effective in training children
454
Apart from nocturesis alarm what can be useful in managing nocturnal enuresis?
= star charts - rewarding positive behaviours
455
For children > 7 with nocturnal enuresis that have not responded to the alarm, what can be prescribed?
= DDACP (synthetic ADH), reduced urine production overnight
456
What is non-accidental injury?
= any physical harm in a child that is deliberately inflicted or results from the failure of a caregiver to prevent such harm
457
What investigations may be done if non-accidental injury is suspected?(2)
- radiology, skeletal survey
- bloods, rule-out clotting disorders or haematological malignancies
458
If non-accidental injury is suspected what is imporant to rule out that would be an alternative cause of bruising? (2)
- clotting disorders
- haematological malignancies
459
Management in suspected non-accidental injury? (5)
- report suspcions to senior
- safeguarding measures to admit child whilst investigations continue
- appropriate medical management
- documentation (clear and thorough)
- social care liaison (to make their own investgation)
460
What is oesophageal atresia?
= refers to congenital disorder in which the oesophagus terminates in a blind-ended pouch rather than connecting to the stomach
461
What is a tracheo-oesophageal fistula?
= an abnormal communication between the trachea and the oesophagus
462
Signs and symptoms of oesophageal atresia? (antenatal 1, postnatal 4)
(antenatal)
- polyhydramnios
(postnatal)
- respiratory distress
- distended abdomen
- choking or problems with swallowing
- difficulty in pass a NG tube
463
Radiological finding in oesophageal atresia (NG tube)
= typically involved in a coiled NG tube on an anterioposterior chest radiograph
464
