Paeds Flashcards
(297 cards)
1
Q
What are the associations of Turner’s syndrome?
A
45XO
Lymphoedema of the hands and feet (as a neonate)
Webbing of the neck
Short stature
Wide-spaced nipples
Congenital heart defects (coarctation of the aorta, aortic stenosis - bicuspid aortic valve)
Delayed or absent puberty
Infertility
Thyroid disorders
Horseshoe kidneys
Coeliac disease
2
Q
How does normal puberty in boys present?
A
Normal puberty starts between 9 and 13 years old
Testicular volume increases above 4ml
There is also penis enlargement, pubic hair growth and a growth spurt
Some boys can also develop transient gynaecomastia during puberty
3
Q
What is congenital adrenal hyperplasia (CAH)?
A
This is due to a 21-hydroxylase deficiency
Infants may present with ambiguous genitalia or bilateral undescended testicles
They are also at risk of a salt-losing adrenal crisis (vomiting, weight loss, floppy unwell infant) at around 1-3 weeks of age + low glucose *Treat with IV dextrose and IV hydrocortisone
It is important to measure urea and electrolytes, as well as a chromosomal analysis and pelvic ultrasound to identify the location of the sex organs
4
Q
How would you differentiate obesity from Cushing’s syndrome?
A
Cushing’s would present with growth failure (so low height but high weight). If a child’s height is above average even if their weight is significantly higher, it could just be obesity
5
Q
How do you calculate predicted height from the parents’ heights?
A
The mean parental height plus 7cm for boys or minus 7cm for girls predicts the adult height of the child ± two standard deviations
6
Q
When is a non-blanching rash unlikely to be meningococcal sepsis?
A
If it is in the superior vena caval distribution AND if the patient has normal observations - usually due to the pressure of coughing
*However ensure this is reviewed by a senior clinician
7
Q
What is the order of management for DKA?
A
Fluid replace (rehydration for 1 hour first) –> IV insulin –> treat underlying cause
*Ensure correction of dehydration and hyperglycaemia occurs slowly over 2 days due to the risk of brainstem demylination with rapid shifts in salts in the cerebrospinal fluid
8
Q
What are signs of life-threatening asthma?
A
Agitation
Cyanosis
Silent chest
Fatigue and drowsiness
Poor respiratory effort
Peal flow <33% of expected
*If by chance you do a blood gas, a sign of life-threatening asthma is hypercapnia causing a respiratory acidosis (you would expected CO2 to be low due to hyperventilation, until they become too tired to ventilate which is when this becomes life-threatening).
9
Q
What are signs of severe asthma?
A
Too breathless to talk or feed
Tachycardia
Tachypnoea
Peak flow between 33 and 55% of expected
10
Q
What are the different types of shock and how do they present?
A
Distributive - shift in fluid to the intrerstitium which results in intravascular hypovolaemia and shock (can cause pulmonary oedema due to the capillary leak of fluid into airway tissues)
Hypovolaemic - due to haemorrhage or dehydration and patient would be pale, cool and poorly perfused
Septic - history of fever suggestive of infection
Cardiac - heart is unable to meet the ciruclatory demands of the body, usually presents with chest pain or other cardiac symptoms. Most common in congenital paediatric cardiac disorders or Kawasaki’s disease
Obstructive - blockage of blood flow from the heart, either due to cardiac tamponande, fluid in the pericardial sac compressing the heart or tension pneumothorax
11
Q
What is the next step in management in status epilepticus after taking two doses of barbiturates?
A
IV/IO phenytoin - you avoid further doses of barbiturates due to respiratory depression
12
Q
How does iron overdose present?
A
Two phases:
1- early vomiting and diarrhoea due to gastric irritation and may present with haematemesis or melaena
24 hour period of improvement
2 - deterioration with liver failure (hypoglycaemia and seizures), drowsiness and coma
13
Q
How does IM adrenaline work?
A
It reduces the capillary leakage which produces airway oedema - it is used in anaphylaxis in a dosage of 1:1000, 250micrograms
*It causes vasoconstriction, bronchodilation and temporarily slows down the anaphylactic process which is histamine-driven
14
Q
What other conditions are associated with Down’s syndrome?
A
Cardiac: VSD, AVSD, ToF
Endocrine: hypothyroidism, Addison’s disease, T1DM
Ocular: cataracts
Malingnancy: leukaemia
Gastrointestinal: duodenal atresia, Hirschprung’s disease
MSK: atlanto-axial instability
*Also increased risk of Alzheimer’s disease in later life
15
Q
How is Prader-Willi inherited?
A
It is inherited by genetic imprinting - where one copy of the gene (either maternal or paternal) is suppressed and the other one is expressed.
In Prader-Willi syndrome, it is the loss of part of the paternal chromosome 15
16
Q
What is galactosaemia?
A
It is due to a deficiency in galactose-1-phosphate uridyl transferase.
it results in illness with lactose-containing milks - there is vomiting, cataracts and recurrent episodes of E.coli sepsis
17
Q
What is the inheritance pattern of Marfan’s syndrome?
A
It is an autosomal dominant condition affecting the fibrillin gene.
It is a connective tissue disorder affecting the MSK, ocular and cardiac systems
18
Q
What is oculocutaneous albinism and what are patients at risk of?
A
It is a metabolic condition affecting the production of skin pigment melanin, to which patients are at risk of skin malignacies especially if there is prolonged sun exposure
19
Q
What are physical features in Down’s syndrome?
A
Hypotonia from birth (centrally and peripherally)
Dysmorphic faces (low set ears, epicanthic folds, protuding tongue, flattened nasal bridge)
Single palmar crease
Sandal gap toes
20
Q
What is respiratory distress syndrome?
A
It is secondary to surfactant deficiency due to the immaturity of type 2 pneumocytes in the alvoeli of the developing lung.
Preterm babies are at risk due to their gestational age. They require artificial surfactant and ventilatory support as their lungs are prone to atelectasis.
*Their lung parenchyma is structurally immature but functional.
21
Q
What is VACTERL syndrome?
A
It is a disorder that affects many body systems:
Vertebral defects
Anorectal atresia
Cardiac defects
Tracheo-oEsophageal fistula
Renal anomalies
Limb abnormalities
22
Q
What are babies with gastroschisis at risk of?
A
They are at risk od dehydration, hypothermia and electrolyte imbalances.
Gastroschisis is when there is a hernation of the bowel through the defect in the anterior abdominal wall of the developing fetus
23
Q
What are causes of asymmetrical IUGR?
A
Placental insufficiency from maternal diabetes and pre-eclampsia
*Asymmetrical IUGR suggests an insult late in pregnancy with head growth sparing
24
Q
What are causes of symmetrical IUGR?
A
Chromosomal anomaly
Maternal smoking
Congenital infection
Maternal alcohol use
*There is low birth weight and head circumference changes (suggests IUGR happened in 1st trimester)
25
What is Wilson's disease?
Autosomal recessive defect in copper metabolism that results in copper deposition in the tissues (especially Kayser-Fleischer rigs in the eye).
It causes liver failure with cirrhosis, neurological sequelae, renal involvement and cardiac complications.
Patients also develop haemolytic anaemia due to copper deposits in the red cell membrane. Thus, patients have a palpable liver and spleen, with anaemia and upper abdominal pain.
26
What is the management for paediatric constipation?
1st line = increase fibre and fluid intake, and exercise
*Consider behavioural change management and toileting using star charts to promote good toileting behaviour
2nd line (if lifetsyle changes do not work or if there is vomiting) = osmotic laxatives (i.e. movicol). If these do not work, then add on a stimulant laxative (senna)
27
Is hepatitis A a notifiable disease?
Yes, to the Health Protection Agency of Public Health England
28
How does autoimmune hepatitis present?
More common in females
Presents between ages 10 to 30 with either chronic liver failure or acute hepatitis
Anti-ANA antibody positive (80% of cases)
Anti-smooth muscle antibody positive (70% of cases)
29
How does pneumonia in children present?
Chest pain
Fever
Upper abdominal pain (in lower lobe pneumonias)
Vomiting due to inflammation of upper airways triggering the gag reflex and increased work of breathing (puts pressure on sotmach just below the diaphragm)
30
Which of the following is not a cause of PR bleeding?
1. Constipation with an anal fissure
2. Intussusception
3. Meckel's diverticulum
4. Bacterial gastroenteritis
5. Adominal migraine
Adominal migraine
Abdominal pain is usually the first presentation of a migraine in children, in the context of a family history of migraine
31
What is the management of Toddler's diarrhoea?
It is a common cause of loose stool in pre-school aged children. It requires no treatment most of the times.
It is due to the immature development of intestinal motility and usually resolves by the age of 5.
Consider loperamide if it becomes socially disruptive to the child and if he becomes unable to attend school.
32
What is choanal atresia?
It is the congenital blockage of the nasal airway which presents with newborn cyanosis and respiratory distress (as children are mostly nasal breahters).
Treatment is surgery under general anaesthesia, where nasal stents are placed in each nostril and held together by stitches.
*Despite being in the name, it has no relation to the anus.
33
What is meconium plug syndrome?
It is a transient immaturity of the gut resulting in failure to move a plug of meconium. It can result from Hirschsprungs disease or cystic fibrosis (causing a meconium ileus). Management is with anal stimulation with a glycerine suppository or a radioopaque contrast enema.
34
Name complications of periorbital cellulitis
Visual loss
Abscess formation
Septicaemia
Orbital cellulitis
Meningitis
35
Oribital cellulitis vs periorbital cellulitis
Periorbital cellulitis involves the area from the skin of the eyelid to the bony area that encloses the eye.
Orbital cellulitis is an infection that involves the eye and the eye structures within the bony cavity of the face.
You can use a CT or MRI but MRI is preferred.
36
What are the complications of congenital cytomegalovirus (CMV) infection?
Sensory-neural hearing loss
IUGR
Hydrocephalus and periventricular calcification
Thrombocytopenia
Microcephaly
Jaundice and blueberry muffin rash
Hepatosplenomegaly
Chorioretinitis --> cataracts
*These usually occur after infection in the first trimester
37
What are febrile seizures?
Generalized seizure that occurs in association with
a fever, in a child with no neurological abnormality.
They are common in children aged 6 months to 6 years. It is thought to occur with the rapid rise in temperature at the star of an infective illness (especially viral)
38
What is erythema multiforme?
Rash described as target lesions with a surrounding red ring
It can be caused by:
drugs (e.g. penicillin)
infection (e.g. atypical pneumonia)
idiopathic
39
What is erythema nodosum?
It is a rash caused by swollen fat under the skin making its appearance red/darker
It can be caused by:
IBD
drugs (e.g. sulphonamides)
infections (e.g. streptococcus)
autoimmune conditions
malignancy
40
What is erythema migrans?
It is a rash seen in Lyme's disease
41
What is erythema toxicum?
It is a benign rash (characteristically small pustules or vesicles surrounded by an erythematous area) seen in newborns in the first 2 weeks of life
42
What is erythema marginatum?
It is seen in rheumatic fever alongside its cardinal symptoms of carditis, arthralgia, subcutaneous nodules, Sydenhma's chorea
43
What is Severe combined immunodeficiency (SCID)?
It is a group of disorders characterised by B and T cell dysfuntion - you will present with low B cells and therefore low immunoglobulins, and low T cells
44
What is hyper IgM syndrome?
As the name describes, there is an overload of IgM antibodies - this is caused by a defect in the CD40 ligand resulting in defective class switching. B cell and T cell numbers are normal.
45
What is DiGeorge syndrome?
It is caused by the microdeletion on chromosome 22q11.2
It manifests as cleft palate, aortic arch and other cardiac abnormalities (ToF, VSD), thymic hypoplasia, hypocalcaemia and learning difficulties
Microdeletions are best detected with FISH (fluorescence in situ hybridisation)
46
What are two important infective complications of eczema?
1. Cellulitis (bacterial superinfection). This is usually caused by gram+ve cocci (staphylococcus and streptococcus) - occurs due to breakage of the skin barrier in the eczematous areas which allows entry of bacteria into the inflamed epidermis and dermis.
2. Eczema hepeticum (herpes simplex virus infection)
47
How can a severe respiratory infection in early childhood later present?
It can present with bronchiectasis - dilation and poor mucociliary clearance with predisposition to further infection
48
When would you suspect *Mycoplasma pneumoniae* instead of *Streptococcus pneumoniae* pneunominal infection in children?
Although *Streptococcus pneumoniae* is the most common typical cause of pneumonia, it is more likely in under 4 year olds. Meanwhilse, *Mycoplasma pneumoniae* would be more common in older children
49
Which of the folllowing is not a complication of primary ciliary dyskinesia (PCD)?
* Pancreatic insufficiency
* Infertility
* Sinusitis
* Bronchiectasis
* Dextrocardia
Pancreatic insufficiency - pancreatic secretions are not reliant on cilia for the expulsion of enzymes and pancreatic fluids. They travel along the pancreatic duct and join biliary secretions at the ampulla of Vater.
PCD affects the respiratory tract and reproductive organs as they are both aligned with cilia to ensure movement of particles.
Cilia is also involved in the determination of sites of internal organs during development - children can present with dextrocardia alone or situs inversus
50
What is chronic lung disease and how does it occur?
Chronic lung disease is by definition an oxygen requirement at 36 weeks corrected gestation or at 28 days post-term.
It occurs in premature babies as a consequence of barotraumas or volutrauma during ventilation, surfactant deficiency and oxygen therapy (thought pathophysiology is complex). It can be minimised by using the lowest possible pressure and volume settings to optimise respiratory function in premature infants.
These children are at risk of respiratory tract infections, particularly RSV for which paluvizumab can be given prophylactically
51
Which lung and which lobe do foreign bodies most likely obstruct?
Right middle lobe - the right main bronchus is the widest, shortest and most vertical of the bronchi, so it is the path of least resistance for the foreign body to descend
52
What does the newborn blood spot screening (NBSS) test detect?
Cystic fibrosis
Congenital hypothyroidism
Sickle cell disease
Severe combined immmunodeficiecny (SCID)
Inherited metabolic diseases (like phenylketonuria or medium chain acyl dehydrogenase deficiency)
53
What best represents respiratory complications of muscular dystrophy?
1. Normal FVC, low FEV1/FVC ratio
2. Flattened diaphragms on CXR
3. Morning dips in peak expiratory flow rate
4. Extrathoracic obstruction on flow-volume loops
5. Reduced FVC, normal FEV1/FVC ratio
Restrictive pattern of respiratory disease (reduced FVC, normal FEV1/FVC ratio). This is due to weak intercostal and diaphragmatic muscles and scoliosis --> reduced capacity for lung and chest wall expansion.
1 and 3 are seen in asthma where there is an obstructive pattern (poor airflow)
2 is seen in hyperinflation and air trapping, for example bronchiolitis
There is no history suggestive of extrathoracic obstruction (3) such as chest pain, cough or lymphadenopathy
54
What type of rib fractures are highly suspicious of non-accidental injury?
Posterior rib fractures
55
What is the most common congenital heart defect?
Ventricular septal defects (VSDs)
The 2nd most common is patent ductus arteriosus
56
What are common symptoms and signs associated with congenital heart disease?
Respiratory distress and sweating with feeds - the effort of feeding tired the child out and burns calories so they may have growth resterictions and failure to thrive
Hepatosplenomegaly can develop secondary to right-sided heart failure
They can also present with cyanosis if there is a right-to-left-shunt
57
What does a child with a history of intercurrent illness suddenly being unwell with tachycardia suggest?
Supraventricular tachycardia (may be caused by Wolff-Parkinson-White syndrome). They would usually present with a narrow complex tachycardia.
SVT is the most common childhood arrhythmia and you would treat by doing:
1. Circulatory and respiratory support
2. Vagal manoeouvres
3. IV adenosine
4. Electrical cardioversion with synchornised DC cardioversion
58
What is Eisenmenger's syndrome?
When a large VSD is left untreated, it can cause high pulmonary flow eventually leading to thickening of the pulmonary artery walls with increased resistance to flow.
Eventually the shunt decreases and the person becomes less sympomatic.
However at around 10-15 years of age, the shunt suddenly reverses due to increased pressures on the right side causing cyanosis. This can cause death by right-sided heart failure by the ages of 40-50,
59
Which is not a feature of cardiac insufficiency?
* Scattered wheeze on auscultation of the chest
* Central cyanosis
* Sacral oedema
* Tachypnoea with the apex beat palpable in the 7th intercostal space just lateral to the mid-clavicular line
* Hepatomegaly
**Central cyanosis** - can be related to other lung pathologies or right-to-left cardiac shunts
Wheeze and bi-basal crackles are due to pulmonary oedema secondary to HF
Sacral oedema - feature of HF if a child is constantly lying down
Tachypnoea and displaced apex beat is due to left ventricular hypertrophy suggestive of HF
Hepatomegaly is due to back pressure in the venous system resulting in congestion of the portal vein in HF
60
Which is not associated with trisomy 21?
* Tetralogy of Fallot
* ASD
* VSD
* AVSD
* Transposition of the great arteries
Transposition of the great arteries
61
What should a septic screen in a child <3months include?
Blood culture
Urine culture
Lumbar puncture
CXR
CRP and other basic bloods
62
What is the triad of nephrotic syndrome?
Proteinuria
Hypoalbuminaemia
Generalised oedema
*In children a common presentation is puffy eyes
63
What are causes of hypertension in children?
Essential hypertension
Renal - renal artery stenosis, chronic kidney disease, Wilm's tumour
Cardiac - coarctation of the aorta
Endocrine - Cushing's syndrome, phaeochromocytoma, neuroblastoma
Metabolic - hyperaldosteronism, congenital adrenal hyperplasia
64
Which of the following is not a complication of Wilm's tumour?
* Hypertension
* Malnutrition
* Renal impairment
* Urinary catecholamines
* Metastatic spread
Urinary catecholamines - these would usually be seen in neuroblastomas (of which catecholarmines would be produced from the adrenal medulla)
65
What are the presenting symptoms of Henoch-Schönlein purpura?
Arthralgia
Rash (urticarial to purpuric)
Abdominal pain
Renal involvement (hypertension, nephrotic syndrome)
It is a common paediatric condition affecting 3-10 year olds, commonly followed by a viral illness - there are GI and renal complications but these are usually benign and self-limiting
66
What are the types of polycystic kidney disease?
Autosomal recessive - presents in childhood with bilateral renal masses, respiratory distress due to pulmonary hypoplasia and congenital hepatic fibrosis with pulmonary hypertension. It is due to tubular dilation of the distal collecting system. Renal function is impaired and progressively deteriorates, requiring renal replacement therapy (dialysis or transplant).
Autosomal dominant - may present in older children or adults. The cysts are grossly dilated nephrons which compress normal renal tissue. It affects renal, hepatic and cerebral vasculature
67
What triggers painful crises in sickle cell disease?
Cold weather
Infection
Hypoxia
Dehydration
68
What is the initial management for painful crises in sickle cell disease?
Analgesia (e.g. oramorph)
Rewarming
Antibiotics
Oxygen
IV fluids
69
What is a common presentation of acute leukaemia?
Limp due to bone pain secondary to dissemination of the disease - do FBC and blood film to look for signs of pancytopenia and the presence of blast cells
70
What should be done before giving steroids for ITP?
Bone marrow aspirate to exclude malignancy (due to risks of further immunosuppression)
71
What are the complications of tumour lysis syndrome and how do you treat it?
Hyperkalaemia
Hyperphosphataemia (which leads to hypocalcaemia)
Gout (hyperuricaemia)
Fluid overload or dehydration
You treat it by giving hyperhydration (with sufficient diuresis to prevent fluid overload), allupurinol, rasburicase and regular monitoring of electrolytes
72
What are the different types of von Willebrand's disease (vWD)?
Type 1 (autosomal dominant) - partial deficiency of vWF
Type 2 (autosomal dominant) - defective vWF
Type 3 (autosomal recessive) - complete deficiency of vWF
73
Why can children present with severe iron deficiency anaemia after 4 months?
Children have sufficient irons tores for 4 months after birth, but after this they require supplementation which usually comes from the introduction of pureed and solid foods.
Hence they can present with microcytic anaemia and low ferritin (iron stores) and low circulating iron. Delayed weaning can also present with hypocalcaemia and poor weight gain.
73
What is a port wine stain?
Also known as naevus flammeus. It presents as a pink-red flat area of erythema with no involvement with local structures.
It is a capillary vascular malformation in the dermis, present from birth and will persist for life. If it is present in the trigeminal distribution, a small proportion of children will have underlying brain involvement (Stuge-Weber syndrome) so an MRI is needed to dectect if the child is at risk of seizures and developmental problems
74
What is the classical picture of infected eczema?
Staphylococcal infection - red inflamed skin with yellow crusts
*Remember to also rule out eczema herpeticum
75
How does a superior vena caval distribution of a capillary rupture present?
Petechiae in the SVC region often caused by raised pressure from coughing or vomiting (which can be caused by coughing)
76
Which of the following is not a cause of erythema nodosum?
* Oral contraception
* Tuberculosis infection
* Hepatitis B infection
* Streptococcal infection
* Sarcoidosis
Hepatitis B infection
*Erythema nodosum are tender red nodules on the shins
77
What is molluscum contagiosum?
It is an infection caused by pox virus and this leads to multiple small pink skin-coloured pearly papules that are umbilicated centrally. Common locations are the chest, abdomen, back, armpits, groin, back of knees
They are usually self-limiting and will self-resolve within a year. Counsel for appropriate hygiene (wear long sleeve clothes).
Occasionally they may need antibiotics following a bacterial superinfection. Chronic papules (>2 years) may need cryotherapy.
78
What is a Mongolian blue spot?
It is a discoloration of the skin (to blue/black) at the base of the spine and on the buttocks. This is more common in darker-skinned races.
They are self-limiting and will fade as the child grows up around the age of 4.
79
What is erythema toxicum?
It is a common innocent rash of the newborn with an erythematous base with small pustules.
It spreads all over the body for the first weeks of life and then fades after a couple of weeks
*Remember to exclude congenital infections
80
What is the aetiology of type 1 diabetes mellitus?
Autoimmune destruction of B cells of the islets of Langerhans in the pancreas
81
What are signs of congenital hypothyroidism?
Usually asymptomatic and identified on screening (raised TSH)
Reduced feeding
Faltering growth
Prolonged jaundice
Constipation
Pale, cold, mottled dry skin
Coarse facies
Large tongue
Hoarse cry
Goitre (occasionally)
Umbilical hernia
Delayed development
82
What is the management for hyperthyroidism?
Hyperthyroidism is due to high pituitary stimulation (high TSH) or endogenous thyroid production (low TSH)
You would manage by giving carbimazole which suppresses thyroxine production. You can also give propanolol for sympotmatic relief (especially tremors).
If these do not work, you would then consider radioisotope treatment and surgery.
83
Which is not a complication of Cushing's syndrome?
* Osteoporosis
* Short stature
* Gastric irritation
* Hypertension
* Hypoglycaemia
Hypoglycaemia - there is actually a tendancy to hyperglycaemia
Other complications include weight gain (truncal obesity and subscapular fat pad), cataracts, striae, thinning of skin with increased bruising, proximal myopathy, increased susceptibility to infection and adrenal suppression
84
What are signs of Klinefelter's?
Delayed puberty with gynaecomastia
Tall stature
Hypogonadism
They are karotypically 47 XXY
85
What is the most common cause of delayed puberty?
Constitutional delay - diagnosis of exclusion
Ensure you exclude hypothalamo-pituitary dysfunction (by measuring GnRH, LH and FSH)
86
What is a greenstick fracture?
A fracture of the long bones in young children where only one cortex is broken and the other is buckled - this is because of young bones being more soft and flexible so a forceful impact will bend the bone rather than break it
87
What is osteogenesis imperfecta?
Also known as brittle bone disease - it is a collagen metabolism disorder (autsomally dominant inheritance).
Common presentation is development of fractures in utero.
Signs and symptoms include: blue sclera, short stature, loose joints, hearing loss, teeth abnormalities, breathing and heart valve problems
88
What is oligoarthritis?
It is an arthritis presentesenting with <5 affected joints in the first 6 weeks - if other joints become affected after, it becomes known as extended oligoarthritis
89
What is psoriatic arthritis?
Interphalangeal joint swelling with scaly skin rash, nail pitting and dactylitis
90
What is polyarticular arthritis?
It presents with symmetrical arthritis of the wrists, hands, ankles and knees - occasionally the spine and jaw may be affected.
It is more common in girls
91
What is enthesitis-related arthritis?
aka juvenile spondylitis - inflammation of enthesesis (areas where tendon and other connecting tissues join to bone)
It presents in older boys with large joint arthritis, swollen tender tendons, sacro-iliitis and bamboo spike on XR.
It is associated with anterior uveitis which if left untreated may cause blindness
92
What is the ideal imaging to diagnose osteomyelitis?
MRI - as it can show acute inflammatory bone changes (with periosteal reactions) and soft tissue swelling - this would only be noticed in an x-ray 2-3 weeks later.
93
What is a strawberry nevus?
Also known as a cavernous haemangioma
These are not present at birth and will typically appear in the first month. They will grow larger before shrinking and disappearing typically before the age of 5. They may compress neighbouring structures as they grow and sometimes ulcerate with troublesome bleeding. They are not flat.
94
What is a capillary haemangiona?
aka "stork bite". This is a pink macule found on the eyelids, central forehead or nape of the neck due to the distension of the dermal capillaries - they mostly fade over the first year.
Those on the neck may persist but will be covered by hair.
95
What is an absence epilepsy?
They usually presence with a brief impairment of consciousness (approx 5-10 seconds)
They have a behavioural arrest and usually stare - can contribute to falling behind on school work.
EEG will show three spike waves per second in all leads
96
What is the difference between breath holding attacks and reflex anoxic seizures?
Breath holding attacks are common in toddlers at times of temper tantrums - the children grow out of them and not treatment is necessary, ensrue you reassure the parents
Reflex anoxic seizures typicall occurs when a child is frightened or hurt (i.e. bump to the head). The child goes pale and faints, hypoxia may cause a short seizure but the child quickly recovers.
97
What are signs of hypoxic ischaemic encephalopathy (HIE)?
What differs mild HIE from moderate HIE?
Signs of HIE include poor feeding, altered tone and seizure activity on EEG - due to hypoxic insult perinatally
Mild HIE presents with irritability, startle responses, poor feeding and hyperventilation
Moderate HIE further has altered tone, reduced movement and seizure activity
98
Which is not a feature of raised ICP?
* Headache
* Morning vomiting
* Sun setting eyes
* Bulging anterior fontanelle
* Papilloedema
Bulging anterior fontanelle - this only occurs in younger children when their fontanelle has not closed, so it provides extra space for the fluid to escape. In older children, this would present as raised ICP
99
Side effects of sodium valproate
Increased appetitie
Weight gain
Hair loss
Liver failure
Teratogenic
100
Side effects of carbamazepine
Lupus erythematous syndrome
Dizziness
Visual disturbances
101
Side effects of lamotrigine
Rash
Behavioural changes
Irritability
102
Side effects of vigabatrin
Behavioural changes
Retinopathy
Sleep disturbance
Weight gain
103
Side effects of levetiracetam (keppra)
Anorexia
Abdominal pain
Vomiting
Diarrhoea
Behavioural changes
Thrombocytopenia
104
What is tuberous sclerosis?
It is a neurocutaneous syndrome usually caused by *de novo* mutations (70% of cases)
Cuteanous features:
* Depigmented 'ash-leaf' patches/amelanotic naevi (Wood's light)
* Shagreen patches (roughened patches of skin over lumbar spine)
* Angiofibromata (in distribution over nose and cheeks)
Neurological features (50%):
* Infantile spasms
* Focal seizures
* Developemntal delay
* Intellectual disability (often with ASD)
Other features:
* Angiomyolipomas
* Subependymal giant cell astrocytomas (which can go on to cause non-communicating hydrocephalus)
105
What are the three complications associated with minimal change disease
Thrombosis - loss of AT-III in the urine
Infection - loss of immunoglobulin in the urine
Hypercholesterolaemia - urinary albumin loss leads to hepatic cholesterol synthesis
106
Indication for USS scan for DDH
At 6 weeks
Breech presentation at 36/40 (regardless of presentation at delivery)
Breech delivery (including <36/40)
Family History of DDH
107
Investigations for DDH
<6m old: Barlow and Ortolani manoeuvre at neonatal screen ± USS (if suspicion remains)
>6m old: X-ray (better than USS at this age)
108
Management for DDH
< 6 months: Pavlik Harness for between 6 to 12 weeks. This keeps hips flexed and abducted with progress being monitored through USS or X-ray
>6 months (or if conservative measures fail, or no progression with harness): Surgery involving close or open reduction, or osteotomy
109
What is Juvenile Idiopathic Arthritis?
Also known as Still's Disease
Most common chronic inflammatory joint disease in children/adolescents in the UK´
Persistent joint swelling (>6w duration) presenting before 16yo, in the absence of infection or other defined cause
110
Signs and symptoms of JIA
Stiffness after periods of rest (gelling) - advised to continue activity as inactivity leads to deconditioning
Intermittent limp
Morning joint stiffness/pain
Limited movement
Later signs:
Inflammation ± bone expansion (overgrowth with leg lengthening/valgus in systemic onset) "Knock Knees"
Salmon-coloured rash (Koebner phenomenon)
Intermittent fever
Visual impairment (chronic anterior uveitis)
Swan-neck deformity and hand problems
111
Management of JIA
Need specialist paediatric rheumatology MDT
Analgesia:
1. Simple e.g. paracetamol
2. NSAID
3. Weak Opioids e.g. codeine
Corticosteroids (as an adjunct):
Intra-articular, oral or IV
Avoided if possible due to risk of growth suppression and osteoporosis
DMARDS:
1. oral or sc methotrexate
2. Sulfasalazine
Biologics / cytokine modulators (i.e. TNF-a inhibitors)
112
What is Osgood-Schlatter Disease (OSD)?
Osteochondritis (inflammation of cartilage or bone) of the patellar tendon insertion at the knee - tibial tuberosity
During growth spurt there may be multiple small avulsion fractures caused by quadricep muscle contractions where they insert into the proximal tibial apophysis
113
Signs and symptoms of OSD
Knee pain after exercise (gradual onset) relieved by rest
Localised tenderness and swelling over tibial tuberosity
Hamstring tightness
114
Management of OSD
Analgesia - paracetamol or NSAIDs
Intermittent application of ice packs over the tibial tuberosity (10-15 mins up to 3 times per day, including after exercise)
Protective knee pads (may relieve pain when kneeling)
Stretching
Reassure that this will resolve over time but may continue until the end of a growth spurt
Advise stopping/reducing all sporting activity (intensity, frequency or duration):
* Could change type of exercise to limit running and jumping requiring powerful quadriceps contraction
* As symptoms decrease, they can gradually increase their exercise levels
* Introduce low-impact quadriceps exercises (e.g. straight leg raises, cycling or swimming)
115
What is osteochondritis dissecans?
Reduced blood flow --> cracks form in the articular cartilage and subchondral bone --> avascular necrosis --> fragmentation of bone and cartilage with free movement of fragments --> activity-related joint pain
116
Management of osteochondritis dissecans
Pain relief (paracetamol or ibuprofen)
Rest and quadriceps exercises
Sometimes surgical intervention is needed (to remove intra-articular loose bodies)
117
What is osteomyelitis?
Infection of the metaphysis of long bones, commonly the distal femur and proximal tibia
* Growth plates in children can prevent further spread into joints
* In infants, before maturation of the growth plates, there can still be possible joint destruction
Often due to haematogenous spread (can be from direct insult) – commonly, staphylococcus aureus
* Normally <5yo
More chronic in onset and less severe than septic arthritis
118
Signs and symptoms of osteomyelitis
Fever
Acute onset limb pain
Immobile limb
Skin swollen, tender and erythematous
119
Management of osteomyelitis
Acute (ABx for 4-6 weeks):
* Flucloxacillin
* If Penicillin-allergic - clindamycin
* If MRSA - vancomycin or teicoplanin
High-dose IV empirical (usually 2-4 weeks) then transition to oral when CRP goes down. Take blood cultures before IV ABx. Surgical debridement may be necessary if there is dead bone or a biofilm
Chronic:
* Clinical assessment, disease staging (Cierny-Mader classification) and optimisation of comorbidities
* Surgical debridement
* IV antibiotics (3 to 6 months)
* Functional rehabilitation
120
What is Perthes' Disease
Avascular necrosis of femoral epiphysis from an interruption of blood supply (osteochondritis). This is followed by revascularisation and re-ossification over 18-36m
121
Signs and symptoms of Perthes' disease
Insidious presentation, limp, hip/knee pain --> limb shortening
122
Management for Perthes' Disease
Conservative management for <6yrs:
* Non-surgical treatment – benign self-limiting condition
* Supportive care for acute pain: simple analgesia, ice packs, protective pad over the tibial tubercle
* Activity continuation
* Physical therapy: stretching of the quadriceps and hamstring muscles, strengthening of the quadriceps, encourage hip abduction
* Education about exacerbations and management
Surgical treatment is reserved for patients who fail to respond to conservative measures (only if >6yrs)
123
What is reactive arthritis?
Transient joint swelling (<6w duration) – often ankles or knees
Follows evidence of extra-articular infection
Low-grade fever
It is a diagnosis of exclusion as there are no +ve findings:
*Bloods (normal or mild elevated CRP)
*Normal XR
124
Causative organisms of reactive arthritis
Preceding a previous infection in the body:
**Enteric bacteria – Salmonella, Shigella, Campylobacter, Yersinia **
Viral infections
STIs (adolescents) – chlamydia, gonococcus
Other – mycoplasma, borrelia burgdorferi / Lyme disease
125
Management of reactive arthritis
No treatment is required as it is self-resolving
Symptomatic relief:
* NSAIDs for pain-relief
* Steroids (severe)
* DMARDs (on-going)
126
What is septic arthritis?
Infection of the synovial joint, usually the hip in 75% of cases
Usually haematogenous spread (may occur following puncture around or infected skin lesions)
RFs: RhA, osteoarthritis, joint prosthesis, crystal arthritis, chronic disease, immunosuppression
Main organism is Staphylococcus aureus
127
Signs and symptoms of septic arthritis
Single joint, usually hip
Erythematous, warm, tender; reduced range of movement; infants will hold limb still (pseudoparalysis) / cry
Acute unwell, febrile child
128
Management of septic arthritis
(IV (2 weeks) --> oral (4 weeks) ABx):
* 1st line: Flucloxacillin
* 2nd line:
* * Penicillin-allergic - Clindamycin
* * MRSA - Vancomycin
* * Gram-negative - 3rd generation cephalosporin (e.g. cefotaxime)
Joint wash-out and aspirated to dryness PRN (through closed needle aspiration or arthroscopically)
Washing out of the joint or surgical drainage may be required
129
What is Slipped Upper/Capital Femoral Epiphysis (SUFE/SCFE)?
Displacement of epiphysis of femoral head postero-inferiorly, requiring prompt treatment to prevent avascular necrosis
* Adolescent (10-15yo during growth spurts); bilateral in 20%
Risk factors = obesity, metabolic endocrine disease (i.e. hypothyroid, hypogonadism), male
130
Signs and symptoms of SUFE/SCFE
Limp/hip pain ± referred to the knee
Insidious or acute onset (i.e. after minor trauma)
“Loss of internal rotation of a flexed hip”
Trendelenburg gait +ve (lean on one side)
131
Investigation findings of SUFE/SCFE
Hip XR in AP and frog-lateral view (both hips):
*** Trethowan sign: line of Klein does not intersect superior femoral epiphyses/asymmetry between line of Klein’s on either side**
* Loss of triangular sign of Capener
* Metaphyseal blanch sign
132
Management of SUFE/SCFE
Ensure patient remains non-weight-bearing, analgesia, immediate orthopaedic referral
Surgical repair
In situ screw fixation across the growth plate
133
What is transient synovitis?
Also known as irritable hip
Occurs in 3-10yo usually and is the commonest cause of hip pain in children
134
Signs and symptoms of transient synovitis
Acute hip pain associated with a viral infection
Low-grade fever
135
Management of transient synovitis
Need to rule of septic arthritis (using Kocher Criteria)
Bed rest
Pain relief can be achieved with paracetamol or NSAIDs
Usually resolves after a few days
136
What is chondromalacia patellae?
Anterior knee pain from degeneration of articular cartilage on posterior surface of patella
Common in young adults (especially female) from overuse in physical activity
137
Signs and symptoms of chondromalacia patellae?
Anterior knee pain
Pain exacerbated by running, climbing stairs or getting up from a chair
Painless passive movements but repeated extension --> pain and grating sensation
138
Treatment for chondromalacia patellae
Physiotherapy to strengthen the quadriceps muscles
139
What is the difference between caput succedaneum and cephalohaematoma?
Caput succeDAYneum: subgaleal bleed which occurs in the subaponeurotic space. Crosses suture lines. Takes days to resolve. Present at birth.
CephalohaeMONTHtoma: between periosteal membrane and cranial bone. Does not cross suture lines. Takes months to resolve. Typically develops hours after birth
140
What is Wilms’ tumour?
Also known as Wilm’s nephroblastoma is a renal malignancy of blastemal, stromal and epithelial cells in children.
It presents with:
Abdominal mass (most common presenting feature)
Painless haematuria
Flank pain
Other features: anorexia, fever
Unilateral in 95% of cases
Metastases are found in 20% of patients (most commonly lung)
141
What is the investigation of choice for reflux nephropathy (secondary to vesicoureteral reflux)?
Micturating cystography (voiding cystourethrogram) to grade the severity
It involves injecting a dye into the bladder via a catheter, and taking x-ray images whilst the child passes urine.
A Dimercaptosuccinic acid (DMSA) scan can follow to identify any renal parenchymal defects.
142
Features of Benign rolandic epilepsy
Partial seizures during sleep
Hemifacial paraesthesias
Oropharyngeal manifestations (e.g. strange noises)
Hypersalivation
May progress to generalised tonic-clonic seizures
143
Features of Juvenile myoclonic epilepsy
Myoclonic and generalised tonic-clonic seizures
When the child is sleep-deprived BUT not during sleep itself
Some children also have absence seizures
144
Features of Temporal lobe epilepsy
Focal temporal lobe seizures can result in deja vu, hallucinations, aphasia and automatism, e.g. lip-smacking
Can progress to generalised tonic-clonic seizures
Don't occur exclusively at night-time.
145
Features of West Syndrome
First year of life
Sudden involuntary contractions of the head/neck/trunk and extensions of the arms/legs
146
Features of Patellar subluxation
Medial knee pain due to lateral subluxation of the patella
Knee may give way
147
Features of Patellar tendonitis
More common in athletic teenage boys
Chronic anterior knee pain that worsens after running
Tender below the patella on examination
148
What causes retinopathy of prematurity?
This occurs in premature babies (<32 weeks) and is. used by over oxygenation (e.g. during ventilation)
This leads to a loss of the red reflex and neovascularisation (proliferation of retinal vessels)
149
What are important congenital infections to remember?
TORCH:
Toxoplasmosis
Other (e.g. syphilis)
Rubella
CMV
HSV
150
What is a physical presentation of spina bifida occulta?
Sacral dimple above nasal cleft or nevus/hairy patch/central pit or discoloured skin
151
What is tethered spinal cord syndrome?
It is a complication of spina bifida occulta where there is fixation of the inelastic tissue of the caudal spine which causes abnormal movement of the spinal cord as the child grows
Symptoms include lower back pain that worsens with activity, gait disturbance, scoliosis, high-arched feet, and neurological dysfunction (e.g. numbness or weakness, bladder and bowel dysfunction).
152
Meningocele vs myelomeningocele?
There are both types of spina bifida
Meningocele = herniation of the meninges between the vertebrae forming a sac that contains CSF - neurology is normal as this sac does not contain neural tissue. It is at risk of rupture which can cause meningitis and hydrocephalus
Myelomeningocele = most severe form of spina bifida where the spinal cord protudes through and opening in the unfused portion of the spinal column. It is associated with paresis, talipes, neuropathic bowel and bladder, and hydrocephalus.
153
How to calculate fluid resuscitation in children?
**1. Emergency resuscitation**
Shocked - 20ml/kg bolus
Not shocked - 10ml/kg
**2. Fluid management (Deficit & Maintenance)**
Deficit: (deficit x weight x 10 - initial bolus if not shocked) over 48hrs
Maintenance: First 10kg = 100ml/kg/day; next 10kg = 50ml/kg/day; every kg above 20kg = 20ml/kg/day
**3. Requirement**
Deficit (- bolus in non-shocked children) + maintenance
154
What is a subgleal haemorrhage?
Rare but serious condition where blood vessels between the scalp and periosteum are damaged - bleed between the aponeurosis of the scalp and the periosteum. It forms a lump that crosses suture lines and it may cause life- threatening blood loss.
It can lead to severe blood loss with life-threatening complications
155
What are features of Marfan's syndrome?
Long, narrow face
High-arched palate
Joint hypermobility
Scoliosis
Flat feet
Pectus excavatum
Associations:
Cardiac - mitral prolapse, aortic aneurysms, aortic dissection
Visual - cataraxcts, lens dislocation
156
How do you treat acute otitis media (AOM)?
Reassurance and pain relief with paracetamol or ibuprofen
Consider antibiotics (5-7 days amoxicillin) if bilateral in children <2yrs, if symptoms do not resolve within 3 days or if co-existing conditions (e.g. CF)
ENT referral if: no response to 2 abx courses, suspected glue ear, recurrent AOM
157
What are indications to admit children with bronchiolitis to hospital?
Apnoea
O2 stats <92%
<50% usual fluid intake
Persistent severe respiratory distress
158
What important chronic condition is a contrindication to the nasal influenza vaccine?
Asthma - consider only the IM influenza vaccine
159
What important allergen can the nasal/IM infleunza vaccine contain?
Egg
160
Notifiable diarrhoeal diseases
Campylobacter jejuni
Listeria monocytogenes
Escherichia coli O157
Shigella
Salmonella
161
Management in nappy rash (irritant dermatitis)
High absorbency nappies
Change nappies ASAP after soiling or wetting
Fragrance-free and alcohol-free wipes
Mild and child asymptomatic: OTC barrier preparations
Moderate: barrier preparation + 1% hydrocortisone cream
Bacterial infection: 7 day oral flucloxacillin
Candidia infection: topical imidazole cream
162
Risk factors for sudden infant death syndrome (SIDS)
Infant:
* Age 1-6 months
* Low birth weight & prematurity
* Male
* Appeared ill in the previous 24hrs
Environment:
* Sleeping prone
* Co-sleeping with parents
* Overheating
* Pillows
Parents:
* Low income
* No maternal educational qualifications
* Poor or overcrowded housing
* Materal age <21yrs
* Smoking or alcohol
163
Features of an atypical UTI
* Poor urine flow
* Abdominal or bladder mass
* Raised creatinine
* Sepsis
* Failure to respond to suitable abx within 48hrs
* Infection with non-e coli organisms
164
Indications for an USS in UTI
During acute infection:
* Atypical UTI signs
* <6 months old with reccurent UTI
Within 6 weeks:
* >6 months old with recurrent UTI
* <6 months old with first-time UTI
165
Indications for a DMSA in UTI 4-6 months after acute infection
Recurrent UTI
or
< 3yrs old with atypical UTI
166
Indications for MCUG in UTI
Children <6 months old with atypical or recurrent UTI
167
What is the treatment for croup?
0.15mg/kg oral dexamethasone
168
What is ringworm?
Unlike what the name suggests, this is actually a fungal infection with an annular (ring-shaped) red, scaly lesion
169
What is the management of ringworm (tinea)?
Mild: topical antifungal cream (terbinafine and clotrimazole)
Moderate: hydrocortisone 1% cream
Severe: 1st - oral terbinafine, 2nd - itraconazole
If involes the scalp (tinea capitits): oral antifungal (e.g. griseofulvin or terbinafine)
*Give treatment to household pets as zoonotic disease
Conservative measures:
* Loose-fitting clothes
* Washing affected areas daily & dry thoroughly
* Avoid scratching
* Frequent washing of clothes and bed linens
* Do not share towels
170
What is quinsy?
This is a peritonsilar abscess which is rare and a serious complication of tonsillitis - an abscess forms between one of the tonsils and the lateral wall of the pharynx
Presentation: severe unilateral throat pain, fever, drolling, foul smelling breath, trismus, changes to voice
171
What is herpangina?
It is similar to hand, foot and mouth disease (also caused by coxsackie virus) with painful mouth and throat ulcers. It causes a more prominent fever and the ulcers are only found on the soft palate, tonsils, uvula and pharynx
172
What prophylactic medication should sickle cell patients receive?
Penicillin V
Folic acid
173
What is the role of hydroxycarbamide in chronic sickle cell disease?
It increases the concentration of foetal haemoglobin.
Though be careful and monitor for any WBC suppression
174
What are two complications of gastroenteritis?
Post-infectious irritable bowel syndrome
Transient lactose intolerance
*They can both present with bloating and diarrhoea when a normal diet is reintroduced - there will be normal blood and stool tests
175
What is the management for enuresis?
<5 years
* Reassurance that it will resolve
>5 years
* 1st line - enuresis alarm with a positive reward system
* 2nd line - Desmopressin
* 3rd line - refer to enuresis clinic + mix of both
176
What is labial fusion?
Benign condition where the labia minora are joined together
Common in girls <7yrs and usually resolves by puberty. It is not present at birth and usually develops at 1-2yrs
Complications: terminal dribbling, increased risk of UTI
Treatment = conservative (resolves by puberty). Topical oestrogen 4-6weeks if symptomatic. Consider surgical sepratation if topical oestrogen is ineffective, fusion is thick and severe, trapped urine causing vulval inflammation
177
What is the metabolic disturbance noted in congenital adrenal hyperplasia?
Metabolic acidosis, hypoglycaemia, hyperkalaemia, hyponatraemia
*"Salt losing crisis" - excretion of sodium for hydrogen ions, with potassium retention
178
What is the triad of shaken baby syndrome?
Subdural haemorrhage
Retinal haemorrhage
Encephalopathy
179
What is the management of GORD in breastfed babies?
1st - breastfeeding assessment
2nd - alginate therapy (1-2 weeks)
3rd - pharmacological
180
What is the management of GORD in formula fed babies?
1st - review feeding history
2nd - smaller, more frequent feeds
3rd - thickened feeds - containing rice starch
4th - alginate therapy
5th - pharmacological
181
What is the pharmacological management of GORD?
GOR:
Gaviscon (alginate therapy)
Omeprazole (PPI)
Ranitidine (histamine antagonist)
*If this does not work and symptoms are severe, consider admission for enteral feeding and Nissen fundoplication
182
How can undiagnosed CF patients present?
Faltering growth
Recurrent chest infections
Rectal prolapse
Nasal polyps
Later: bronchiectasis, diabetes mellitus, cirrhosis, sterility in males
183
What are the main features of CF and treatment?
Respiratory:
Features - chronic cough, recurrent chest infections
Treatment - physiotherapy, mucoactive agents, abx prophylaxis
Gastrointestinal:
Features - pancreatic exocrine insufficiency, malasborption, constipation
Treatment - vitamin supplementation, high-calorie diet, laxatives
Infection:
Features - recurrent infection
Treatment - abx prophylaxis, vaccination
Psychiatric:
Features - depression, anxiety
Treatment - CAMHS
184
What is the management of anaphylaxis?
FLACOS:
Fluids
Legs elevated
Adrenaline
Chlorphenamine
Oxygen
Salbutamol (if wheezy or known asthmatic)
185
What is a technitium-99m scan?
Nuclear imaging that can highlight gastric mucosa such as recurrent intussusception caused by Meckel's diverticulum
186
How does beta thalassaemia present?
Major: Homozygous form with no beta chain production and absolute deficiency of HbA. Profuse anaemia at 3-6 months with growth faltering. There is also signs of extramedullary haemopoiesis such as maxillary overgrowth and frontal bossing - patients are dependent on transfusions
Trait (minor): heterozygous form that presents with well-tolerated hypochromic, microcytic anaemia picked up on routine blood tests
187
What differentiates beta thalassaemia trait from iron deficiency anaemia on a blood test?
Ferritin in normal in beta thalassaemia trait but low in iron defiency anaemia
188
What is the pathophysiology of congenital hypothyroidism?
Thyroid dysgenesis - absent or underdeveloped thyroid gland
Thyroid dyshormonogenesis - disruption of thyroid hormone production
189
Signs of congenital hypothyroidism
Excessive sleepiness
Hypotonia
Constipation
Poor feeding
Jaundice
Macroglossia
Umbilical hernia
190
Which of the following physiological changes in the circulatory system occurs immediately after birth?
1. Closure of the ductus arteriosus
2. Opening of the foramen ovale
3. Increase in venous return to the right atrium
4. Increase in left atrial pressure
5. Increase in pulmonary resistance
**Increase in left atrial pressure** - increased pulmonary flow causes an increase return of blood from the lungs to the left atrium
Closure of ductus arteriosus only occurs hours or days post-birth
There is actually decreased venous return to the right atrium as there is a disconnection from the placenta.
191
How to manage obesity in children?
Increase physical activity to at least 60 minutes per day
Dietary interventions to maintain an energy deficit
Behavioural interventions - realistic goals and rewards
Drug treatments (orlistat) but only recommended in severe cases and in children abover 12yo
192
What is rolandic epilepsy?
Also known as childhood epilepsy with centrotemporal spikes are caracterised by focal seizures that occur at night with abnormal feelings in the face and mouth
193
What is premature thelarche and how do you manage it?
Development of breast tissue in females aged 6 months to 8 years in the absence of other signs of puberty such as pubic hair and growth spurts.
It is a non-progressive, benign condition without complications - parents can be reasured and followed up in 4-6 months to assess for signs of precocious puberty
*In normal puberty there will be an increase in breast size witin 4-6 months of the first palpable breast disc appearing however in premature thelarche, there is little/no change and the breast can become smaller
194
Describe clubfoot
Also known as talipes equinovarus.
The forefoot is adducted (facing downwards and inwards) wilst the sole of the foot faces backwards (hindfoot varus)
195
What are the characteristics of Edwards syndrome?
Micrognathia
Microcephaly with a prominent occiput
Dysplastic ears
Long fingers that overlap
Rocker-bottom feet
Shield chest
196
What are the main physical malformations of foetal alcohol syndrome?
Microcephaly
Short nose
Thin upper lip
Epicanthal folds
Smooth philtrum
197
What can be seen on blood film during EBV infection?
In children >12yo and in the 2nd week of infection, blood film can show >20% atypical lymphocytes
198
How does choanal atresia present?
Children ar eobligate nose breaters so it can manifest as cyanosis or choking when a baby is feeding (oral route is obstructed).
This is releived on crying due to the relieving of the airway obstruction
199
Is school exclusion required during head lice treatment?
No
200
Treatment for head lice
If live head lice are found, start with wet combing (5 times over 3 weeks).
If lice still found at final wet combing, then consider physical insecticides (e.g. dimeticone 4%) to suffocate or chemical insecticides (e,.g. malathion 0.5%) to poison the lice - application is twice, at least 7 days apart
201
What medications can be used to help with the following symptoms in cerebral palsy?
* Muscle stiffness
* Trouble sleeping
* Constipation
* Excessive drooling
Muscle stiffness - baclofen
Trouble sleeping - melatonin
Constipation - laxatives
Excessive drooling - anticholinergics
202
What is a sign of extramedullary haemopoiesis?
Maxillary overgrowth
Frontal bossing
203
What are features of effusion on otoscopy?
Loss of light reflex
Opacification of the drum
Air-fluid level behind the drum
204
What is the management of otitis media with effusion (glue ear)?
This is not an active infection so you would actively observe during 6-12 weeks as spontaneous resolution is common
During this period they should also have 2 hearing tests: pure tone audiometry and tympanometry.
If it persists refer them to ENT for consideration of non-surgical management (hearing aids, active monitoring for 3 months, auto-inflation) or surgical management (myringotomy and grommets)
205
What is milia?
Tiny white cysts that contain keratin and sebum which appear as white dots
It is a common finding in newborns and resolves within a few weeks without treatment
206
What conditions can cause macrocephaly?
Fragile X syndrome
Neuofibromatosis type 1
Tuberous sclerosis
207
What are the characteristics of fragile x syndrome?
Elongated and narrow face
Large protruding ears
Prominent lower jaw and forehead
Macroorchidism
Increased joint problems
Autism spectrum disorder
Intellectual disability
208
What are long-term potential complications of meningitis?
Hearing loss —> audiological assessment
Neurological/developmental problems
Orthopaedic, skin and psychosocial complications
Renal failure
Purpura fulminans
209
What is the choice of fluid hydration in DKA?
0.9% sodium chloride with 40mmol/L potassium chloride until plasma glucose is <14mmol/L at which 5% dextrose is added.
Insulin of 0.05 or 0.1 units/kg/hour is started 1-2 hours post-fluids
210
What is omphalitis?
Redness and swelling around the umbilical stump (before it falls off at 1-2 weeks post-birth).
There may be pus coming out of the umbilical cord with systemic symptoms
Low threshold for intervention due to rapid progression to sepsis - perform blood cultures and give IV ABx if suspected
211
What is Blount’s disease?
Abnormality of the medial aspect of the proximal tibial growth plate which causes slow and progressive bowing (varus)
Two types:
Infant - affects children up to 3yrs, is bilateral and involves the tibia
Adolescent - affects children over 10yrs, is usually unilateral and involves both the tibia and femur
*In children <2yrs, a bowing can be physiological as genu varum
212
What is the newborn hearing test and when is it carried out?
Automated otoscoustic emission test at 4-5 weeks. This involves an earpiece to play gentle clicking sounds and the detect picks up low-intensity sounds produced by the cochlea.
If there is a hearing problem, the automated auditory brain stem response test is done.
213
When is the pure tone audiometry test done?
At 4-5 years of age before school starts
214
What problems can phimosis cause?
Balanoposthitis - inflammation of the glans and foreskin due to colonisation of organisms (e.g. Candida and bacteria)
Urinary problems - dysuria, retention and difficult urination
Paraphimosis
UTIs
215
What should you never do in phimosis?
Manually retraction of the foreskin - is painful and can cause scarring
216
What are the 3 main components that lead to the development of acne?
Increased sebum production (normal in puberty)
Presence of Cutibacterium acne’s
Blocked pores
217
What is the management of acne?
1st line (topical): benzoyl peroxide, adapalene ot azelaic acid
*Benzoyl peroxide - reduces sebum production, dries skin and bacteriostatic effect on C. acne’s
*Adapalene - topical retinoids with exfoliating effect (reduces blocked pores) and anti-inflammatory effect on sebaceous glands affected by C. acne’s
2nd line (oral antibiotics): max 3 months of lymecycline or doxycycline.
3rd line (after 2 failed ABx courses): refer to dermatology for isotretinoin
*In females, the COCP can be an alternative to Abx
218
What is Alport syndrome?
X-linked recessive disorder of type IV collagen with a triad of:
Nephritic syndrome
Sensorineural deafness
Ocular disorders
219
What is infantile seborrhoeic dermatitis?
AKA cradle cap
It affects areas rich in sebaceous glands such as the scalp or face. The rash is often described as being greasy with yellow scales. It is not itchy and infants are otherwise well.
It is self-limiting and resolves within several months.
Consider conservative treatments like emollients or gentle shampooing. If it is extensive, you can consider hydrocortisone 1%.
220
What should children aged >3months with acute pyelonephritis or upper UTI receive?
7-10 day course of oral cephalexin or oral co-amoxiclav
221
What should children >3months with cystitis/lower UTI receive?
3 day course of oral trimethoprim or oral nitrofurantoin.
2nd line ABx is amoxicillin
222
What is tic disorder?
Fast, repetitive muscle movements that result in sudden and difficult to control body jolts or sounds
Common, appear around 5yo, not usually serious, most disappear by adulthood
* May come and go over several years
* 1 in 3 rules (1 in 3 won’t have tics as an adult, 1 in 3 have mild tics, 1 in 3 will have severe tics)
223
What is the treatment for tic disorder?
Conservative:
* Sleep & stress management, don’t draw attention to tic, don’t tell child off for it
* Habit reversal therapy
* Exposure with response prevention
Medication:
* 1st line- anti-psychotics (neuroleptics) e.g. risperidone
* 2nd line - clonidine (alpha-2 agonist). This can treat ADHD at the same time
* You can also consider Clonazepam, Tetrabenazine (treats tics that are caused by an underlying condition i.e. Huntington’s), Botulinum toxin
Surgery:
* Deep brain stimulation therapy (for severe Tourette’s syndrome)
224
What are the three characteristics of Tourette’s syndrome?
* Tics must begin before 18yo
* Must persist for longer than 1 year
* Chronic and multiple tics with no cure
*Remember to exclude any other cause
225
What is Pityriasis rosea?
Pityriasis rosea presents with oval-shaped, scaly patches often distributed in a ‘fir-tree’ pattern. It is often preceded by a larger herald patch (seen toward the bottom of the image). There is often no prodrome and the rash is self-limiting.
226
What is Pityriasis versicolor?
Pityriasis versicolor is a superficial fungal infection caused by Malassezia furfur. It presents as scaly patches which vary in colour (hence versicolor) from hypopigmented, brown or pink. Classically the patches are more noticeable following a sun tan.
227
What is the treatment of infantile hydrocele?
They usually resolve within the first few months of life but can be considered for surgical repair if still present at 1-2 years of age.
228
What is the treatment of scabies?
Permethrin (topical insecticide) - applied to the whole body and allowed to dry before being washed off 8-12 hours later. A second application must be done one week after the first.
Bedding and clothing should be washed at high temperatures.
229
What is the most common heart defect in Down's syndrome?
AVSD
230
What mutation is associated with retinoblastoma?
RB1 gene on chromosome 13
231
What does a Wilm's tumour on MRI show?
Intrinsic renal mass
232
What mode of bilirubin measurement is done in patients:
<24 hours old
24-2 weeks old
<24 hours old - serum
24-2 weeks old - transcutaneous
233
What is the FeverPAIN score?
Fever in past 24 hours +1
Absence of cough or coryza +1
Symptom onset three or less days +1
Purulent tonsils +1
Severe tonsil inflammation +1
Score <2 likely not bacterial and no abx needed
Score 2-3 potentially bacterial with a delayed abx prescription considered
Score 4-5 bacterial infection and abx should be considered immediately
234
Why should amoxicillin be avoided in patients with a sore throat?
It can cause an itchy and widespread maculopapular rash in those whose pharyngitis is caused by EBV
235
What scoring system can be used in croup?
Westley Croup Severity Score
236
What is the management of Crohn's disease?
Induce remission:
* Nutritional management is effective in 85-100% patients (crohns.org)
* Replace diet with **whole protein modular diet** – excessively liquid, for 6-8 weeks
* May need NG if the child struggles to drink that much
* Products are easily digested, provide all nutrients needed to replace lost weight
* Pharmacological management: steroids (prednisolone) may be used to induce remission
Maintain remission – you can use steroids, but these have long-term consequences…
* Aminosalicylates (e.g. mesalazine)
* Immunosuppressive drugs (azathioprine, methotrexate, mercaptopurine)
* Azathioprine cannot be given to people with a TPMT mutation
* Must not have live vaccines
* Must have pneumococcal and influenza vaccines
* Anti-TNF antibodies in biologic therapies (e.g. infliximab, adalinumab)
237
What is the remission management of ulcerative colitis?
1st line: topical or oral aminosalicylates – if no improvement 4 weeks after starting, move to oral, then 2nd line
* Often used to maintain remission
* Can use oral azathioprine or mercaptopurine if aminosalicylates insufficient
2nd line: topical or oral corticosteroid (i.e. if aminosalicylates not tolerated/contraindicated)
* Prednisolone
* Beclomethasone
3rd line: oral tacrolimus
4th line: biological agents (infliximab, adalimumab and golimumab)
5th line (resistant disease) --> surgery (colectomy with ileostomy or ileojejunal pouch)
238
What is alopecia areata?
Presents with patchy areas of complete hair loss in children with no other symptoms - can have some onycholysis and pitting.
In most cases hair will regrow within 1 year without intervention
239
What is ophthalmia neonatorum?
Inflammation of the conjunctiva within the first 28 days of life.
Often benign and spontaneously resolves after careful cleaning with saline
However, if bacterial infection (presence of mucopurulent apearance) is suspected, babies require same-day referral to an opthalmologist. Treat using oral erythromycin for 14 days.
240
What is the first line medication for the following seizures?
* Tonic-clonic
* Myoclonic
* Absence
* Focal
* Tonic-clonic - sodium valproate
* Myoclonic - sodium valproate
* Absence - ethosuximide or sodium valproate
* Focal - carbamazepine or lamotrigine
241
What investigations can be done when suspecting a Wilms' tumour?
Abdominal USS with doppler - identifies heterogenic mass and rules out involvement of the renal vein and inferior vena cava
CT/MRI - used for staging of the tumour
242
What is the gold-standard investigation for diagnosing cerebral palsy?
MRI - you can see periventricular leucomalacia
243
What is the management for meconium aspiration?
Examine the oral cavity and remove any meconium with a large-bore suction catheter
Abx treatment = IV gentamicin and ampicillin
244
What is otitis externa?
Outer ear infection which presents with otalgia and a red and itchy ear which may have dry skin.
It is usually due to a bacterial infection (e.g. S. aureus or P. aeruginosa) and may be precipitated by children sticking dirty fingers into their ears. Sometimes, otitis externa is due to seborrheic dermatitis or contact dermatitis and there may be yellow, oily scales
245
How do you treat otitis externa?
Topical antibiotic with or without a steroid.
If symptoms persist, oral antibiotics and/or topical antifungals may be considered.
246
When are cleft lip and cleft palates repaired?
Cleft lip - first 3 months of life
Cleft palate - between 6-12 months old
247
What are poor prognostic factors of ALL?
* T cell surface markers
* age <2 or >10 years
* Male
* WCC > 20x10^9/L
* non-Caucasian race
248
What is used to diagnose asthma?
FEV1 with bronchodilator therapy reversilibulity (expect at least 12%)
249
What is the definition of a hydrocele?
A collection of fluid within the tunica vaginalis. It can occur in neonates due to failure of obliteration of the processus vaginalis
250
What is the definition of a varicocele?
Dilations of the pampiniform plexus that have a pathognomonic ‘bag of worms’ feeling to them. This may be related to valvular incompetence and usually occur in adolescents
251
What is the genetic inheritance of achondroplasia?
Autosomal dominant but 70% are sporadic mutations in the fibroblast growth factor receptor 3 (FGFR3)
252
What are physical signs of achondroplasia?
Main:
Short stature with shortening of limbs
Lumbar lordosis
Trident hands
Others:
Large heard, frontal bossing
Short, broad hands
Depression of nasal bridge
253
What is the cause of infantile torticollis and what is the management?
In most cases, infant torticollis is caused by a sternocleidomastoid tumour and is congenital, muscular torticollis. This would mean that the baby’s range of motion in their neck is limited and they will struggle to turn their head in one direction. This may manifest subtly, such as them only managing to feed successfully from one breast. On examination, you may feel a mobile, non-tender nodule in the body of the sternocleidomastoid muscle. This is rarely firm or tender.
Mx: stretching and strengthening exercises especially during “tummy time”. Physiotherapy may be required and, very rarely, surgery can be considered.
254
What are associated conditions that can cause Talipes equinovarus?
Spina bifida
Edward syndrome
Oligohydramnios
Arthrogryposis multiplex congenita
Cerebral palsy
255
What is Prehn's sign and what does it suggest?
Prehn’s sign refers to relief of scrotal pain upon elevation of the testicles
negative = testicular torsion
positive = epididymitis
256
What are the classical signs you see in testicular torsion?
Pitting at the testicular base (positive Ger sign)
Testicles in a horizontal lie (positive Brunzel sign)
Abnormal elevation of testes (positive Deming sign)
No nodule (negative blue-dot sign)
No pain relief on elevation (negative Prehn’s sign)
257
What are examples of secondary lesions in acnes?
Secondary lesions are those which occur as a result of the primary lesions healing.
For example, excoriations from spots that have been picked or macules. Macules can be erythematous (red marks, usually on fair skin) or pigmented (dark marks, usually on darker skin).
258
What is the most common brain tumour in children?
Pilocytic astrocytoma, which is a type of glioma. It is usually benign and has a 90% cure rate with surgical removal and chemotherapy if required.
Rosenthal fibres are the histological hallmark of pilocytic astrocytomas.
259
What is the treatment of balanitis?
Conservative: hygiene advice and slaine washes (including under foreskin)
Medical: 1% hydrocortisone cream. If fungal - clotrimazole cream. If bacterial - flucloxacillin.
*Consider swabs in unknown cause
260
What is the incubation and infectious period for the following:
Mumps
Measles
Rubella
**Mumps**
Incubation: 15-24 days
Infectious period: 5 days before to 5 days after parotid swelling
**Measles**
Incubation: 10-14 days
Infectious period: 4 days before to 4 days after rash
**Rubella**
Incubation: 14-21 days
Infectious period: 7 days before to 4 days after rash
261
What are the associations of neurofibromatosis type 1 and 2?
Type 1:
* Axillary freckling
* Lisch nodules (hamartomas of the iris)
* cafe-au lait spots
* Phaeochromocytomas,
* Neurofibromas
* Bone deformities (e.g. scoliosis)
Type 2:
* Bilateral acoustic neuromas +- deafness
* Meningiomas
* Ependymomas
262
What is osteogenesis imperfecta?
What are the values for calcium, phosphate, PTH and ALP?
AKA brittle bone disease = rare group of genetic disorders affecting collagen metabolism which leads to fragile bones that break easily. In addition to fractures following minor trauma, children may have blue sclera and conductive, mixed or sensorineural deafness. Teeth may also be affected.
It tends to be associated with normal biochemical results (like osteoporosis)
263
What are expected endoscopy results for Crohn's and UC?
Crohn's:
Skip lesions, strictures - mouth to anus, especially terminal ileum
UC:
Continuous distribution with no strictures - rectum and colon ± backwash ileitis
264
What are expected biopsy results for Crohn's and UC?
Crohn's:
Transmural, deep ulceration, cobblestone mucosa, fibrosis, granulomas, fistulae
UC:
Mucosal lesions, crypt abscesses, shallow/broad ulceration, pseudopolyps
265
What is the definition of recurrent tonsilitis?
* More than 7 episodes per year for 1 year
* 5 episodes per year for 2 years
* 3 episodes per year for 3 years
Should be referred to an ENT specialist for consideration of tonsillectomy.
266
What is chronic suppurative otitis media?
Chronic inflammation of the middle ear and mastoid cavity --> tympanic membrane perforation with subsequent ottorhoea and bacterial infection.
A visible defect may be seen in the tympanic membrane and, sometimes, a cholesteatoma. A CT or MRI scan may be considered if complications are suspected (e.g. meningitis, intracranial abscess, facial nerve palsy).
Conservative measures (rinsing of the ear, topical antibiotic and steroid drops) or surgical treatment (tympanoplasty with insertion of graft) should be considered.
267
What are the most common organisms that cause pneumonia in children?
Neonates
Infants and Young Children
Children >5 years
Neonate: Group B Streptococcus, Gram-negative Enterococcus
Infants and Young Children: Streptococcus pneumoniae or Haemophilus influenzae
Children > 5 years: Mycoplasma pneumoniae, Streptococcus pneumoniae and Chlamydia pneumoniae
268
How do you treat mild UC affecting the rectum (proctitis)?
Topical (i.e. per rectal) 5-ASAs such as mesalazine
269
What abx is used against Haemophilus influenzae B?
Ceftriaxone (IV)
270
What is the treatment of viral warts?
Salicylic acid
271
What is the management for cow's milk protein allergy?
1st - trial cows' milk elimination from diet for 2-6 weeks:
* Breastfed babies - mother to exclude cow's milk protein from diet. Consider daily supplement of 1g of calcium and 10 mcg of vitamin D. NB it takes 2-3 weeks to fully eliminate cow’s milk from breastmilk
* Formula-fed babies - replacement of cows' milk-based formula with hypoallergenic infant formula (e.g. extensively hydrolysed formula or amino acid formula if severe)
* Weaned infants/older children - exclue cow's milk protein from diet
2nd - regular growth monitoring, with nutritional counselling from dietician
3rd - re-evaluate tolerance every 6-12months. Reintroduce with milk ladder
272
What is the definition of a complex febrile seizure?
>15 min
Focal
Repeat within 24 hours
Repeat in same illness
No recovery <1hr
*risk 4-12% of subsequent epilepsy
273
What would the HPG axis blood tests show for:
Kallman syndrome
Turner's syndrome
Fragile X syndrome
Kallman syndrome (hypogonadotropic hypogonadism) - low GnRH, low FSH and LH, low oestrogen
Turner's syndrome (primary hypogonadism) - low oestrogen leads to elevated LH and FSH from a loss of negative feedback
Fragile X syndrome (premature ovarian failure) - low oestrogen and raised LH and FSH from a loss of negative feedback
274
What is the most common arrhythmia in children?
What ECG changes would you see?
Supraventricular tachycardia (SVT)
Narrow complex tachycardia (short PR interval), delta waves (in WPW), T wave inversion (due to ischaemia)
275
What is the management of SVT?
1. Circulatory and respiratory support: Tissue acidosis corrected + Positive pressure ventilation if needed
2. Vagal stimulating manoeuvres (e.g. carotid sinus massage, cold ice pack to face, blowing into a syringe) - 80% success
3. IV adenosine (induces AV lock after rapid bolus infusion - terminates tachycardia)
4. Electrical cardioversion with synchronised DC shock
276
What abx do you give in suspected bacterial meningitis?
< 3 months old: IV cefotaxime (or ceftriaxone) + amoxicillin/ampicillin
> 3 months old: IV ceftriaxone
277
What are the X-ray features seen in Rickets?
Thickened and widened epiphysis
Cupping metaphysis
Bowing diaphysis
278
What are the different causes of Rickets?
Nutritional (primary)
Intestinal malabsorption
Defective production of 25-hydroxyvitamin D
Increased breakdown of 25-hydroxyvitamin D
Defective production of 1,25-dihydroxyvitamin D
Resistance to effect of 1,15-dihydroxyvitamin D
Hypophosphataemic rickets
| Page 226 Lissaurs of specific causes
279
What is Guttate psoriasis and what triggers it?
Small lesions (approx 1cm in diameter) that are raised and scaly, pink/red colour.
It is triggered by a bacterial or viral infection, commonly streptococcus pharyngitis.
280
What is Peutz-Jegher syndrome?
Autosomal dominant disease with development of hamartomas throughout the GIT. Predominately involves the small bowel but large bowel and rectum can be involved. Polyps can start presenting from the age of 10
Key distinguishing feature from other colonic polyp disorders are hyperpigmented spots on the lips, hands and genitalia.
There is an increased risk of breast and GI cancers.
281
After what age would you be concerned about strabismus?
Strabismus = where eyes point in different directions.
This is common up to 3 months of age due to refractive effors (corneal refelctions would be seen in different positions). If it is persistent >3 months old, then a specialist opthalmology referral is needed
282
Boy born at 40 weeks with male phenotype, but neither testicle can be palpated on examination. What is the most likely diagnosis?
a. Androgen insensitivity syndrome
b. Excess in utero oestrogen exposure
c. Klinefelter’s syndrome
d. Physiological delay in descent
e. Retractile testes
Physiological delay in descent - occurs in 3-5% of full-term male infants
283
What investigations do you do in biliary atresia?
USS: triangle cord sign
Cholescintigraphy/TIBIDA isotope scan (gold-standard)
ERCP +- biopsy (diagnostic)
284
What do you do when you suspect eczema herpticum?
Immediate referral to A&E
285
Child has symptoms of perennial rhinitis (blocked nose, nasal voice, running eyes). What is the most likely causative allergen in this scenario?
House dust mites
286
What is the treatment for cellulitis?
Flucloxacillin.
However, if infection is near the eyes or nose, give co-amoxiclav
287
Mother with SLE has a baby who is born with a slow heartbeat. What is the most likely diagnosis?
Congenital heart block
It can occur as a result of autoantibodies that the mother produces in response to SLE, which can cross the placenta and affect the developing baby. These autoantibodies specifically target the heart's electrical conduction system, leading to impaired signal transmission and a slow heartbeat in the fetus.
288
What are causes of neonatal conjunctivitis?
<48hrs: gonococcus
1-2 weeks: chlamydia trichomatas
289
What can be used as prophylaxis for migraines?
Topiramate or Propanolol
290
What are the different forms of management of impetigo?
Localised, non-bullous: topical hydrogen peroxide 1% cream --> topical fusidic acid (2%)
Widespread, non-bullous: oral flucloxacillin or topical fusidic acid
Bullous, systemically unwell: oral flucloxacillin
*Ensure good hygiene measurements + school exclusion until lesions are crusted or until 48hrs post-abx
291
What route and where should the influenza vaccine be administered for children with egg allergies?
The influenza vaccine is given nasally to most children
The nasal spray flu vaccine has a low egg content and is safe to give in school or in a clinic to children who do not have a serious egg allergy.
Children who have previously needed treatment in intensive care for an egg allergy may be offered the nasal spray vaccine in hospital.
292
What is the gold standard test to diagnose precocious puberty?
Gonadotrophin stimulation test
293
What can be given if a child is too unwell to receive oral dexamethasone in croup?
Nebulised budesonide or IM dexamethasone whilst awaiting hospital admission
In severe cases, you can consider nebulised adrenaline
294
Where does brain damage occur in the different subtypes of cerebral palsy?
Spastic - pyramidal tracts
Dyskinetic - basal ganglia
Ataxic - cerebellum
295
How should low birth weight babies (<2500g) be fed according to the WHO?
Exclusive breastmilk for the first 6 months. If the mother is unable to produce, then donor breast milk should be sought
296
What are complications of using a Pavlick Harness to treat DDH?
Avascular necrosis and temporary femoral nerve palsy
