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?
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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?
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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
