Day 8 PAEDS Flashcards
What do NICE say about chickpox?
NICE Clinical Knowledge Summaries state the following:
Advise that the most infectious period is 1–2 days before the rash appears, but infectivity continues until all the lesions are dry and have crusted over (usually about 5 days after the onset of the rash).
A newborn is due for her hearing screening test.
She was born at 36 weeks with no complications during the pregnancy, via a normal vaginal delivery.
Which of the following tests is most appropriate to use in a child of this age?
Otoacoustic emission test is used to screen newborns for hearing problems
Which tool is used to screen newborns’ hearing?
Otoacoustic emission test is used to screen newborns for hearing problems
A 14-month-old child presents to you in primary care after a convulsion.
The parents are very distressed as an uncle has epilepsy and they are concerned their daughter may have it.
The child appears alert with a temperature of 38.4C, something which the parents believe she has had for four days.
Previously, calpol has helped bring this down from a high of 40.7ºC.
You also note a pink, maculopapular rash on the chest with minimal spread to the limbs, something which mum says she noticed this morning.
The child has been feeding but has had some diarrhoea and you feel some enlarged glands on the back of her head.
There is no rash in the mouth.
Given your findings, what do you feel is the most likely underlying cause of the child’s symptoms?
Roseola infantum is caused by Herpes virus 6.
It is characterised by a 3-5 day high fever followed by a 2 day maculopapular rash which starts on the chest and spreads to the limbs.
This generally occurs as the fever is disappearing.
Which virus is associated with Kaposis’ sarcoma?
Herpes virus 8 is associated with Kaposi’s sarcoma and is most commonly seen in AIDS patients.
Which disease is associated with Herpes virus 8?
Herpes virus 8 is associated with Kaposi’s sarcoma and is most commonly seen in AIDS patients.
Which disease does Strep A cause?
Group A Streptococcus tends to cause infections of the throat (aka strep throat) and skin (such as cellulitis, erysipelas and impetigo).
Which bacterial infection is associated with nfections of the throat (aka strep throat) and skin (such as cellulitis, erysipelas and impetigo)?
Group A Streptococcus tends to cause infections of the throat (aka strep throat) and skin (such as cellulitis, erysipelas and impetigo).
Key features of Roseola
Features
- high fever: lasting a few days, followed later by a
- maculopapular rash
- Nagayama spots: papular enanthem on the uvula and soft palate
- febrile convulsions occur in around 10-15%
- diarrhoea and cough are also commonly seen
A 10-month-old infant is brought to accident and emergency with a 3 day history of fever and a new onset rash affecting the arms, legs and abdomen that began today. Despite this fever the child has been his usual self and does not seem to be irritated by the rash.
On closer inspection, the rash appears erythematous with small bumps that are merging together. None of the lesions have scabbed over. The rash is predominantly on the limbs and there are no signs of excoriation (skin picking).
The child is now afebrile at 36.9ºC.
Bearing in mind the likely diagnosis, what is the most likely causative organism?
Roseola infantum is a common viral illness that causes a characteristic 3 day fever and then emergence of a maculopapular rash on the 4th day, following the resolution of the fever.
How does Roseola differ from Varicella?
(3)
Roseola rash appears on arms and trunk
Varicella rash is widespread and disseminated
Additionally, Varicella is very itchy, Roseola is not.
What is the causative agent of Roseola?
Which part of the body does it attack?
Caused by HHV6
Attacks nerve cells - rare complication is encephalitis
How does Neisseria infection present?
(4)
Neisseria meningitides is a common cause of bacterial meningitis which presents with symptoms of meningism (photophobia, stiff neck, headache) +/- non-blanching rash seen with meningococcal septicaemia.
A 12-year-old boy presents for review. He was diagnosed with asthma three years ago by his general practitioner.
He is currently on a salbutamol inhaler which he is using 2 puffs 3 times daily, a paediatric low-dose beclomethasone inhaler and oral montelukast.
He still has a night time cough and has to use his blue inhaler most days.
Unfortunately, there appears to have been little benefit following the addition of montelukast. His chest is clear on examination today with no wheeze and a near-normal peak flow.
What is the next step in his management?
Stop montelukast and add salmeterol
salmeratol is a long acting beta agonist
How does montelukast work?
(2)
Blocks the action of leukotriene D4 in the lungs resulting in decreased inflammation and relaxation of smooth muscle.
A 35-year-old pregnant woman presents with premature labour at 30 weeks gestation.
What is the most important treatment for the prevention of neonatal respiratory distress syndrome?
Administer dexamethasone to the mother
What are tocolytics?
When would they be used?
Name 3 examples
Tocolytics are agents that can be used to suppress pre-term labour, however they are not routinely used.
Since administration of maternal steroids takes one to two days to increase surfactant levels, tocolytics can be considered in certain situations to buy time.
magnesium sulfate (MgSO4), indomethacin, and nifedipine
A 15-year-old teenage girl presents with delayed puberty, having not commenced her menses. She is well in her self generally, with no significant medical history.
On examination, she is of slim build, with small breasts. There is no pubic hair present. Her abdomen is soft and non-tender, though there are marble-sized groin swellings bilaterally.
What is the most likely explanation for this presentation?
(4)
Androgen insensitivity - classic presentation is ‘primary amenorrhoea’
The key symptom here is the groin swellings, which combined with ‘primary amenorrhoea’ and no pubic hair points towards a diagnosis of androgen insensitivity (previously testicular feminisation syndrome).
The groin swellings here are undescended testes. This is a condition in which the patient is genetically male (46XY), but phenotypically female.
Feminisation is a result of increased oestradiol levels, which lead to breast development.
What is the genotype of Klinefelter’s syndrome?
What are the features of Klinefelter’s? (6)
How is it diagnosed?
Klinefelter’s syndrome is associated with karyotype 47, XXY
Features
- often taller than average
- lack of secondary sexual characteristics
- small, firm testes
- infertile
- gynaecomastia - increased incidence of breast cancer
- elevated gonadotrophin levels
Diagnosis is by chromosomal analysis
What is Kallman’s syndrome?
What is its inheritance?
How does is it thought to develop?
Kallman’s syndrome is a recognised cause of delayed puberty secondary to hypogonadotrophic hypogonadism.
It is usually inherited as an X-linked recessive trait.
Kallman’s syndrome is thought to be caused by failure of GnRH-secreting neurons to migrate to the hypothalamus.
Features of Kallman’s syndrome
Features
- ‘delayed puberty’
- hypogonadism, cryptorchidism
- anosmia
- sex hormone levels are low
- LH, FSH levels are inappropriately low/normal
- patients are typically of normal or above average height
Cleft lip/palate and visual/hearing defects are also seen in some patients
What is Androgen insensitivity syndrome?
What is the genotype?
Androgen insensitivity syndrome is an X-linked recessive condition due to end-organ resistance to testosterone causing genotypically male children (46XY) to have a female phenotype.
Management of androgen insensitivity syndrome
(3)
counselling - raise child as female
bilateral orchidectomy (increased risk of testicular cancer due to undescended testes)
oestrogen therapy
A baby is 12 hours old and was born at term. The mother had gestational diabetes during her pregnancy.
The mother has chosen to formula feed exclusively.
The baby is currently comfortable on the postnatal ward, and her latest capillary blood glucose reading is 2.3mmol/L.
The examination is normal.
What would be the next step in management?
Neonatal hypoglycaemia: if asymptomatic then encourage normal feeds and monitor glucose
An 18-month old child presents to the GP with his worried parents. They have just noticed that his foreskin swells when he urinates and they cannot retract the foreskin. Which of the following is the best immediate management of this condition?
Reassure parents and review in 6-months
In children less than 2 years of age, phimosis (a non-retractable foreskin) is normal and will most likely resolve with time
A 15-year-old girl comes into the GP practice requesting contraception. You counsel her and together decide the implant would be the best method for her.
You feel that she has the capacity to make this decision and consent to the insertion.
However, in previous consultations, you have found her not to be capacitous for certain decisions and have involved her parents.
Which of the following is required, according to the GMC, in order to continue with the insertion of the implant?
Just the patient’s consent
Capacity is time and decision dependent. If in the past the patient has not had capacity to make a decision, but today for this decision you feel she has, the only consent you need is from her.
A male infant is born prematurely at 34 weeks gestation by emergency cesarean section.
He initially appears to be stable. However, over the ensuing 24 hours he develops worsening neurological function.
Which processes is most likely to have occurred?
(4)
Intraventricular haemorrhage is a haemorrhage that occurs into the ventricular system of the brain.
In premature neonates it may occur spontaneously.
The blood may clot and occlude CSF flow, hydrocephalus may result.
In neonatal practice the vast majority of IVH occur in the first 72 hours after birth, the aetiology is not well understood and it is suggested to occur as a result of birth trauma combined with cellular hypoxia, together the with the delicate neonatal CNS.
How is neonatal IVH managed?
(2)
Is largely supportive, therapies such as intraventricular thrombolysis and prophylactic CSF drainage have been trialled and not demonstrated to show benefit.
Hydrocephalus and rising ICP is an indication for shunting.
You are working on the paediatric ward and are called to see a child who the nurses have noticed is persistently ‘floppy’.
You assess the child and find no acute cause for concern and wonder whether there might be an underlying issue.
Which of the following is most likely to be the underlying cause of neonatal hypotonia in this case?
Neonatal hypotonia: associated with Prader-Willi
Causes of neonatal hypotonia (4)
neonatal sepsis
Werdnig-Hoffman disease (spinal muscular atrophy type 1)
hypothyroidism
Prader-Willi
A couple attend the GP practice seeking advice as they are currently pregnant with their first son.
The father suffers from haemophilia A and is worried about the risk of passing this disease onto his son.
The mother is not a carrier and has no family history of any disorders.
What is the percentage chance of the baby inheriting haemophilia A?
Haemophilia A is an x-link recessive condition
There is no male-to-male transmission in X-linked recessive conditions
Features of Perthe’s disease
(4)
Perthes’ disease is a degenerative condition affecting the hip joints of children, typically between the ages of 4-8 years
hip pain: develops progressively over a few weeks
stiffness and reduced range of hip movement
x-ray: early changes include widening of joint space, later changes include decreased femoral head size/flattening
premature fusion of the growth plates
Management of Perthe’s disease
(4)
To keep the femoral head within the acetabulum: cast, braces
If less than 6 years: observation
Older: surgical management with moderate results
Operate on severe deformities
A six-week-old infant is brought into the emergency department with a history of poor feeding for 4 days and increasing difficulty in breathing.
There is no significant medical history.
On examination the infant has a blue tinge to the lips and a ejection systolic murmur on auscultation of the precordium.
What is the most likely underlying diagnosis?
Cyanotic congenital heart disease presenting at 1-2 months of age is TOF
Cyanotic congenital heart disease presenting within the first days of life is TGA.
A 24-year-old nulliparous woman presents in spontaneous labour and you are involved in the vaginal delivery of a baby boy weighing 3.6 kg.
An Apgar score is used to asses the health of the newborn.
Which of the following contains the correct components of the Apgar score?
The components of the Apgar score include
pulse,
respiratory effort,
colour,
muscle tone
reflex irritability.
You are shadowing a doctor working on the labour ward who is asked to review a 2-hour-old delivered at 41+2 weeks by ventouse as the mother is anxious about the appearance of her baby’s head.
On examination, you see a soft, puffy occipital swelling with some light bruising from the ventouse cup, the swelling appears to cross the suture lines.
The baby seems well in herself otherwise and the neonatal hearing screen which occurred earlier that morning was unremarkable.
What is the likely cause for this appearance?
Caput succedaneum is a puffy swelling that usually occurs over the presenting part and crosses suture lines
Parents bring their 4 week old formula fed infant to the short stay paediatric ward.
They are concerned because he has persistent non-bilious vomiting and is becoming increasingly lethargic.
Despite this, his appetite is substantial.
On examination, he appears pale and you can see visible peristalsis in the left upper quadrant.
What is the most likely diagnosis?
This presentation should raise the suspicion of pyloric stenosis.
Visible peristalsis can be visualised as the stomach tries to push its contents past the obstruction.
The vomiting is non-bilious as the level of obstruction is proximal to the second part of the duodenum where bile enters the gastrointestinal tract.
This is contrast to malrotation and duodenal atresia.
You have just assisted with the normal vaginal delivery of a baby girl, during the delivery there was a large amount of meconium.
On observation of the baby just after the birth the presence of which of the signs would prompt you to call the neonatal team?
(7)
As per the NICE guidelines if any of the following are observed after any degree of meconium, then baby must be assessed by the neonatal team;
- respiratory rate above 60 per minute
- the presence of grunting
- heart rate below 100 or above 160 beats/minute
- capillary refill time above 3 seconds
- temperature of 38°C or above, or 37.5°C on 2 occasions 30 minutes apart
- oxygen saturation below 95%
- presence of central cyanosis
A concerned mother brings her 4-year-old son to see the GP, worried about his walking and balance. The child learned to walk around 2 years old, much the same as his older sister.
However, over the last few months, his mum has noticed that he has become reluctant to walk and often trips or falls when he does.
On examination, the child is slim built but has disproportionately large calves. When asked to walk across the room he does so on his tiptoes. Gowers test is positive.
What investigation is considered most appropriate to confirm the likely diagnosis?
Duchenne muscular dystrophy
genetic testing rather than a muscle biopsy is now used to make a diagnosis
What is Gower’s test?
To get up from a sitting or supine position, the child must first become prone on the elbows and knees. Next, the knees and elbows are extended to raise the body.
What is the inheritance pattern of DMD
Duchenne muscular dystrophy is an X-linked recessive inherited disorder in the dystrophin genes required for normal muscular function.
Investigations in DMD (2)
raised creatinine kinase
genetic testing has now replaced muscle biopsy as the way to obtain a definitive diagnosis
Chickenpox is a risk factor for invasive group A streptococcal soft tissue infections including necrotizing fasciitis
