Paediatrics Flashcards
Diagnostic criteria for Kawasaki’s:
Criteria for diagnosis of Kawasaki disease include high-grade fevers for >5 days, and 4/5 of the ‘CREAM’ features:
Conjunctivitis (bilateral, non-exudative)
Rash (any non-bullous rash)
Edema/Erythema of hands and feet
Adenopathy (cervical, commonly unilateral and non-tender)
Mucosal involvement (strawberry tongue, oral fissures etc)
In addition to the above clinical features, children with Kawasaki are typically very unwell and often ‘flat’ - far more so than with most other febrile illnesses they have.
Kawasaki’s treatment:
IVIg and high-dose aspirin
Surveillance of coronary artery aneurysms via echocardiograms
Recovery from the acute episode can take weeks
What is the major complication of Kawasaki’s?
Main concern is of coronary aneurysms and all patients suspected of kawasaki should have an urgent echocardiogram.
Explain what kawasaki’s is:
Kawasaki disease is a type of vasculitis which is predominately seen in children. Whilst Kawasaki disease is uncommon it is important to recognise as it may cause potentially serious complications, including coronary artery aneurysms.
Summarise Klinefelter’s syndrome:
Klinefelter syndrome only occurs in males and is often associated with HYPOGONADISM, INFERTILITY, a TALL stature and somewhat feminised appearance
Classic signs of Turner syndrome:
Short stature
Recurrent otitis media
Spoon shaped nails - often seen due to lymphoedema.
History of intussusception is a contraindication for what vaccine?
Rotavirus
Children with immunosuppression should not receive what vaccines?
Live attenuated vacines including MMR.
Presentation of Wilms tumour:
Wilm’s tumours typically do not cross the midline but in up to 5% of cases they may be bilateral. It presents with a palpable abdominal mass, distension and haematuria.
How does Lumacaftor work?
Lumacaftor increases the number of CFTR proteins transported to the cell surface.
What is cystic fibrosis?
Cystic fibrosis (CF) is an autosomal recessive disorder causing increased viscosity of secretions (e.g. lungs and pancreas). It is due to a defect in the cystic fibrosis transmembrane conductance regulator gene (CFTR), which codes a cAMP-regulated chloride channel.
What are the features of cystic fibrosis?
The features of cystic fibrosis include neonatal period (around 20%): meconium ileus, less commonly prolonged jaundice; recurrent chest infections (40%); malabsorption (30%): steatorrhoea, failure to thrive; other features (10%): liver disease. 5% of patients are diagnosed after 18 years and may have symptoms such as short stature, diabetes mellitus, delayed puberty, rectal prolapse, nasal polyps, male infertility/female subfertility.
What organisms colonize cystic fibrosis patients?
- Staphylococcus aureus
- Pseudomonas aeruginosa
- Burkholderia cepacia
- Aspergillus
Diagnosis of CF?
Cystic Fibrosis can be diagnosed by neonatal heel prick day between day 5 and day 9, sweat test, faecal elastase, and genetic screening.
Causes of false positive sweat test?
skim
Causes of a false positive sweat test include malnutrition, adrenal insufficiency, glycogen storage diseases, nephrogenic diabetes insipidus, hypothyroidism, hypoparathyroidism, G6PD, and ectodermal dysplasia.
Most common reason for a false negative sweat test?
skim
The most common reason for false negative sweat tests is skin edema, often due to hypoalbuminemia/hypoproteinemia secondary to pancreatic exocrine insufficiency.
Management for CF?
Management for cystic fibrosis includes twice daily chest physiotherapy, high calorie diet with high fat intake, vitamin supplementation, pancreatic enzyme supplements taken with meals, lung transplant, and Lumacaftor / Ivacaftor used to treat cystic fibrosis patients who are homozygous for the delta F508 mutation.
Skim the pathophysiology of CF:
CFTR proteins are responsible for transporting chloride out of cells. In the airway, the CFTR proteins transport chloride ions into the mucus which attracts water molecules making it less viscous. In cystic fibrosis, this protein is defective, thus no chloride ions are transported into the mucus making it thick and sticky and hard to clear.
What does a sweat test show in someone with CF?
Patients with cystic fibrosis have abnormally high sweat chloride. The normal value is < 40 mEq/l, and cystic fibrosis is indicated by > 60 mEq/l.
Neonatal features of CF?
The neonatal features of Cystic Fibrosis include failure to thrive, meconium ileus, and rectal prolapse.
What are the respiratory features of Cystic Fibrosis?
The respiratory features of Cystic Fibrosis include chronic sinusitis, nasal polyps, cough, wheeze, hemoptysis, recurrent lower respiratory tract infections, bronchiectasis, pneumothorax, cor pulmonale, and respiratory failure.
What are the gastrointestinal complications of cystic fibrosis?
The gastrointestinal features of Cystic Fibrosis include pancreatic insufficiency resulting in diabetes mellitus and steatorrhea, cirrhosis, portal hypertension, gallstones, and distal intestinal obstruction syndrome.
Bedside investigations for CF?
CF tests:
Resp = Sputum culture or throat swab, spirometry.
GI = Vitamin A, D, E, K
Diabetes = glucose levels, glucose tolerance test
Bloods = FBC, urea and electrolytes, liver function tests, clotting studies.
Aspergillus skin prick test or serology.
Conservative management of CF:
Conservative management for Cystic Fibrosis includes education about the condition, fertility and genetic counseling, dietician, psychosocial counseling, chest physiotherapy, and screening for complications of Cystic Fibrosis such as osteoporosis.
What is medical management for Cystic Fibrosis?
Medical management for Cystic Fibrosis includes treating infective exacerbations with antibiotics, nebulized mucolytics, and bronchodilators.
Treating pancreatic insufficiency with insulin replacement regime, exocrine enzymatic replacement, and vitamin A, D, E, K.
Treating abnormal liver function/deranged liver function tests with Ursodeoxycholic acid.
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Treating worsening progressive lung disease with oxygen, non-invasive ventilation, and diuretics if signs of cor pulmonale.
Diagnosis:
Infant less than 3 months old bouts of excessive crying and pulling-up of the legs, often worse in the evening.
Infantile colic
Young adult with hypertension, systolic murmur = ?
+ weak posterior tibial and dorsalis pedis pulses.
Coarctation of the aorta
What is included in the 6-in-1 vaccine?
Diphtheria, tetanus, pertussis, polio and haemophilus influenzae type b, hepatitis B.
Childhood infections:
The prodrome is characterised by fever, irritability and conjunctivitis =
Measles
How does the chicken-pox rash change over time?
Itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular.
Fever, malaise, muscular pain
Parotitis (‘earache’, ‘pain on eating’): unilateral initially then becomes bilateral in 70%
Diagnosis?
Mumps
Rash: pink maculopapular, initially on face before spreading to whole body, usually fades by the 3-5 day
Lymphadenopathy: suboccipital and postauricular.
Diagnosis?
Rubella
Koplik spots: white spots (‘grain of salt’) on buccal mucosa
Rash: starts behind ears then to whole body, discrete maculopapular rash becoming blotchy & confluent.
Diagnosis?
Measles
Lethargy, fever, headache
‘Slapped-cheek’ rash spreading to proximal arms and extensor surfaces.
Diagnosis?
Erythema infectiosum aka slapped cheek syndrome
Erythema infectiosum is caused by what pathogen?
Parvovirus 19
Pathology of scarlet fever?
Reaction to erythrogenic toxins produced by Group A haemolytic streptococci.
Presentation of scarlet fever:
Fever, malaise, tonsillitis
‘Strawberry’ tongue
Rash - fine punctate erythema sparing the area around the mouth (circumoral pallor)
Describe perfectly the rash seen in scarlet fever:
Rash - fine punctate erythema sparing the area around the mouth (circumoral pallor).
Mild systemic upset: sore throat, fever
Vesicles in the mouth and on the palms and soles of the feet.
Diagnosis?
Hand foot and mouth disease
Hand foot and mouth disease is caused by what?
Caused by the coxsackie A16 virus.
Most common causative organism of tonsilitis?
Strep pyogenes
When is a squint in a baby a red flag?
If still present at 8 weeks
What is Perthe’s disease?
Perthes’ disease is a degenerative condition affecting the hip joints of children, typically between the ages of 4-8 years. It is due to avascular necrosis of the femoral head, specifically the femoral epiphysis. Impaired blood supply to the femoral head causes bone infarction.
What x-ray changes are seen in Perthe’s disease?
x-ray: early changes include widening of joint space, later changes include decreased femoral head size/flattening.
Management of Perthe’s disease
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
What x-ray finding is seen in Ricketts?
Widening of joints, specifically of the wrists, due to an excess of non-mineralised osteoid at the growth plate.
Treatmet for threadworm in child older than 6 months
mebendazole is used first-line for children > 6 months old. A single dose is given unless infestation persists
TTP (thrombotic thrombocytopenic purpura) FATRN
TTP presents with: Fever, anaemia (haemolytic), thrombocytopenia. Renal, Neuro.
How does coarctation of the aorta present?
This is a congenital narrowing of the aorta and would present with absent/weak femoral pulses; hypertension in arms but hypotension in legs.
Antibiotics for children with meningitis
< 3 months: IV amoxicillin (or ampicillin) + IV cefotaxime
> 3 months: IV cefotaxime (or ceftriaxone)
The most common complication of measles is what?
Otitis media
Diagnosis of Meckel’s diverticulum
Technetium scan
Diagnosis of intussuception and what is seen?
An abdominal ultrasound will show classic ‘target’ sign (concentric echogenic and hypoechogenic bands) and can also show complications of disease such as free-abdominal air or presence of gangrene.
Official heart defects in Turner’s syndrome:
Bicuspid aortic valve (most common)
Aortic root dilatation
Coarctation of the aorta
How to differentiate Kawasaki disease from Scarlet fever?
Kawasaki doesnt respond well to antipyretic, has non-tender lymphadenopathy, and desquamates in the palms and soles. Kawasaki’s also affects the lips.
Scarlet fever responds well to antipyretics, has tender lymph nodes, and desquamates on the chest and back.
Evidence of bowel sounds in a respiratory exam of a neonate in respiratory distress should make you consider what?
Diaphragmatic hernia
Infants with pertussis may present with apnoeas rather than the classic whoop
Infants with pertussis may present with apnoeas rather than the classic whoop
What respiratory condition in kids is a notifiable disease
Whooping cough
Jaundice, appetite and growth disturbance in the first few weeks of life think…
Biliary atresia
A child with scarlet fever can return to school how long after commencing antibiotics?
24 hours
Symptoms of refeeding syndrome:
Symptoms of refeeding syndrome may include oedema, confusion and tachycardia. Blood tests initially show hypophosphataemia and it is treated with phosphate supplementation..
Chest x-ray in a child with transient tachypnoea shows what?
Chest x-ray in transient tachypnoea of the newborn may show hyperinflation and fluid in the horizontal fissure.
- this commonly occurs in caesarean babies as the lung fluid doesn’t get squeezed out.
Roseola infantum is caused by what?
Herpes virus 6
How does roseola infantum aka HHV-6 present?
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
What murmur do you hear in Turner’s syndrome?
- what’s the cause?
Turner’s syndrome is associated with an ejection systolic murmur due to bicuspid aortic valve.
Croup treatment
Oral dexamethasone to be taken immediately regardless of severity.
A baby is born with micrognathia, low-set ears, rocker bottom feet and overlapping of fingers. Diagnosis?
Edward’s syndrome
Breech baby what investigation is needed?
Pelvic Ultrasound at 6 weeks to look for DDH
‘High grade fever’ child then goes on to develop a rash.
- diagnosis
Roseola infant
- HIGH fever 2. Rash
Rachitic rosary with genu varum
Rickets
High fever lasting >5 days, red palms with desquamation and strawberry tongue are indicative of
Kawasaki disease
Neonate with feeding intolerance, abdominal distension and bloody stools, which can quickly progress to abdominal discolouration.
What are you worried about?
Necrotising enterocolitis
Consider a diagnosis of pneumonia if the child has:
high fever (over 39°C) and/or
persistently focal crackles.
Consider a diagnosis of pneumonia if the child has:
high fever (over 39°C) and/or
persistently focal crackles.
Explain the significance of an early hand preference in paeds?
- when?
Hand preference before 12 months is abnormal - it could be an indicator of cerebral palsy.
Newborn has heel prick showing raised immunoreactive trypsinogen.
- Thoughts and what’s your follow up?
Raised immunoreactive trypsinogen (IRT) result is positive in CF.
Follow up with a sweat test, which will be high if they have CF.
Trypsinogen is an enzyme produced in the pancreas.
Kawasaki desquamates where?
Palms and soles
What fever + rash is ibuprofen contra-indicated for children?
Chicken-pox
Abx of choice for whooping cough
Whooping cough - azithromycin or clarithromycin if the onset of cough is within the previous 21 days
Eczema affects where in infants?
In infants, atopic eczema usually involves the face and extensor surfaces of the body as opposed to its classical flexural distribution.
How to remember the gross motor milestones easy:
(3) Head, (6) Shoulders, (9) Knees, and (12) Toes.
Coeliacs 1st line testS
NICE also advises that IgA and IgA tTG antibody testing is the first line test of choice.
How does Henoch-Schonlein purpura classically present?
Henoch-Schonlein purpura classically presents with abdominal pain, arthritis, haematuria and a purpuric rash over the buttocks and extensor surfaces of arms and legs
Duchennes - cardiac pathology?
Dilated cardiomyopathy
