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469 Cards in this Set
- Front
- Back
an IgE food allergy which results in swelling of the face and vomiting is classified as?
|
'moderate'
|
|
why is the airway threatened in severe food allergies?
|
the tongue swells up (angioedema) blocking the airway
|
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when is the most common time for children to present with IgE food allergies?
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80% will present in the first year of life
|
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what food allergy is associated with atopic dermatitis?
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IgE mediated food allergy (35% of moderate AD have IgE-mediated FA)
|
|
what is the prevalence of food allergy?
|
3-9%
|
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IgE mediated allergies occur when T-cells communicate with what type of cell?
|
B-cells, which make IgE!
|
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what type of cells is IgE bound to, which creates an allergic reaction?
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basophil/mast cells
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what are the typical symptoms of IgE mediated food allergy?
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sneezing, watery eyes, angioedema, itching, urticaria
|
|
what the concordance of peanut allergy in twins?
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MZ: 67%. DZ: 7%
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which is a greater risk factor for food allergy: parent or sibling with atopy?
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Sibling (10-fold), vs parent (4-fold)
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what are the most common culprits of IgE mediated food allergies?
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milk, eggs, wheat, peanuts, shellfish, fish, soy, tree nuts, seeds
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what is a 'positive' reading on skin prick testing?
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>3mm
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are skin prick tests highly specific? Highly sensitive?
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SP (no!) 30-60%; SN (yes!) 75-95%
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T or F: skin prick reaction size is predictive of the reaction severity?
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No, it is only predictive of the likelihood of a reaction
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With a positive skin prick test, what else is required for a diagnosis of 'allergy'?
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A positive clinical history
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what IgE allergens are commonly outgrown?
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milk, egg, soy, wheat (80% will outgrow each of these)
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who reacts more severely to an allergen, older or younger patients?
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older
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what are some predictors of a severe reacttion to an allergen?
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a) older child, b) dose of allergen, c) asthma, d) nuts/fish/shellfish, e) epitope type
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anaphylaxis is a multisystem reaction, which involves which organ systems?
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Respiratory and/or Cardiovascular
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non-IgE mediated is when memory T-cells are made to an antigen if no suppression from?
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T3 cells
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what chemicals are released in a non-IgE mediated allergic reaction?
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cytokines
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what time frame after ingestion does a non-IgE mediated allergic reaction occur?
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2-24 hours
|
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what is the best test for non-IgE food allergies?
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food elimination, followed by rechallenge
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what are the presenting features for eosinophilic esophagitis?
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vomiting, food refusal, dysphagia, abdominal pain, bolus obstruction
|
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what are the gross findings of EoE on endoscopy?
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visible strictures, oedematous/furrowed mucosa, pale white patches
|
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how is eosinophilic esophagitis treated?
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modify diet; swallow inhaled fluticasone
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what are the four components of a history, in determing if one has a food allergy?
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determine the (1) symptoms attributed & the (2) time course in which they happen. (3) determine if reproducible & if (4) any improvement following elimination
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What part of the airway is hyperresponsive in asthma?
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bronchioles
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what percent of Australian children have asthma?
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10-15%
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asthma cause how many deaths per year?
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250,000
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what two infections contribute to acute asthma in children?
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RSV, rhinovirus
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what percent of children with Asthma are atopic?
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80%
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a child is known to have a recurrent wheeze that remits. Is this asthma?
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no
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how would you assess the severity of asthma to be, in a child with a drowsy state of consciousness & able to speak in only words?
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severe
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what pulse rate is indicative of a severe asthma attack?
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>200bpm
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what is the oxygen saturation of a child with a moderate asthma attack? Heart rate?
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O2sat: 90-94%. Pulse: 100-200
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if central cyanosis is present, what is the severity of this asthma attack?
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severe/life-threatening
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what is the intensity of a wheeze in a moderate asthma attack?
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moderate to loud
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what is the intensity of a wheeze in a life threatening asthma attack?
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often quiet
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what is the PEF in a moderate asthma attack?
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40-60%
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what is the FEV1 in a moderate asthma attack?
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40-60%
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with a spacer, what is the dosing of ventolin in a child < 20kg?
|
6 puffs (600mcg). [double to 1200mcg, if > 20kg]
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how often should ventolin be administered to an asthmatic child before they are discharged home?
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q3hr
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what is the dose of salbutamol given for acute asthma, if nebulized, in a child < 20kg?
|
2.5mg [double to 5.0mg in a child > 20kg]
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what is the dosing of atrovent given to a < 20kg child, with a spacer?
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4 puffs q6h [double to 8 puffs if > 20kg]
|
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what is the trade name for ipatropium?
|
Atrovent
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what is the trade name for salbutamol?
|
Ventolin
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what is the dosing for ipatropium for a < 20kg child, if delivered by nebulizer?
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250mcg q20 x3, then q6h [if >20kg, double dose to 500mcg]
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if a child is hospitalized for acute asthma, what dosing of oral prednisone is given?
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day 1 - 2mg/kg (up to 60mg); day 2-4+ - 1mg/kg
|
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how long is the course of IV steroids for acute asthma, in children?
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24-48 hours
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what is the IV dose of methylprednisolone for acute asthma in children?
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1mg/kg q6-8h
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what is the IV dose of hydrocortisone for acute asthma in children?
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4mg/kg q4-6h
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what severity of asthma attack should receive ipatropium?
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moderate
|
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when is magnesium used in asthma attacks?
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severe attacks (by 10mg/kg bolus)
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when is intravenous salbutamol used in asthma attacks?
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in severe attacks, with a poor response to nebulized salbutamol
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if a child has nighttime asthma syptoms twice per month, how severe is their asthma? Frequently?
|
not every week: mild. Frequent: severe.
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describe 'frequent intermittent' asthma?
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no symptoms between exacerbations; exacerbations >2/month; >80% predicted PEF or FEV1
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a PEF or FEV1 of 60-80% predicated is classified as what severity of asthma?
|
moderate
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what percent of asthma cases are 'infrequent intermittent'?
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75%
|
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what preventer medication should be given to children with frequent intermittent asthma?
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montelukast, inhaled cromones, or low-dose ICS
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what severity of asthma warrants commencing ICS or montelukast as a preventer?
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frequent intermittent, or worse (persistant mild/mod/sev)
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what initial dose of fluticasone should be used for an asthmatic commencing ICS?
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100-200ug/day
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if inhaled corticosteroids prove inadequate for controlling persistent asthma symptoms, what can be changed in medication?
|
ICS can be increased, or LABA or montelukast can be added
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what are some inhaled corticosteroids commonly used in Australia? (and what are their trade names?)
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Fluticasone (Flixotide), Budenoside (Pulmicort), Beclomethasone (Qvar), Ciclesonide (Alvesco)
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what are the two ICS/LABA combos available in Australia? (and what do they contain?)
|
Seretide (fluticasone + salmeterol), Symbicort (budenoside + eformoterol)
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which asthma preventers are prescribed BID?
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Flixotide, Pulmicort, Qvar, Seretide & Symbicort
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what is the trade name for the asthma preventer Sodium Cromoglycate? How often is it administered?
|
Intal Forte, prescribed TID
|
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what questions should be explored to assess asthma control, at a checkup?
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1) symptoms (day/night), 2) freq. of reliever, 3) freq. of exacerbations, 4) limitations to physical activity, 5) lung function
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what are the details of an Asthma Action Plan?
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a) regular use of preventers, b) when to use relievers, c) when to use oral steroids, d) when to seek medical attention
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at what age can a child start using a large spacer?
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>4 years of age
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at what age can a child start using a MDI without a spacer?
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>8 year of age
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what is the 4x4x4 asthma first aid plan?
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1) sit person upright, 2) 4 puffs of reliever, 3) wait 4 minutes, 4) rpt 2-3, call ambulance if no improvement
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what clinical features would make one think of malignancy in a child?
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fever, weight loss, pain > physical findings, night pain, hepatosplenomegaly, lymphadenopathy, anaemia, metaphyseal lucencies (X-ray)
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what is the definition of arthritis?
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1) joint swelling/effusion OR, 2) two or more: tenderness/pain on movement, decreased ROM, increased warmth
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what diagnostic test is used for Juvenille Idiopathic Arthritis?
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None; this is a diagnosis of exclusion from other forms of arthritis, for individuals < 16yrs & pain > 6 wks
|
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what infectious agent causes a bright red facial rash, and is associated with painful, swollen joints of the hand and feet?
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parvovirus (parvovirus arthritis)
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what is the most likely causative organism for arthritis, when proceeded by sore throat and fever 2 weeks prior?
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Group A Streptococcus (GAS: Acute rheumatic fever)
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Subcut nodules, Pancarditis, Arthritis, Chorea, & Erythema marginatum are the major features of what infection?
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Acute Rheumatic Fever
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Subcut nodules, Pancarditis, Arthritis, Chorea, Erythema marginatum are the major features of Acute Rheumatic Fever. What are the minor features?
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a) Fever, b) Arthralgia, c) inc ESR/CRP, d) prolonged PR interval
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What organism would one expect to find in joint aspirate, in post-infectious/reactive arthritis?
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None; joint fluid will be sterile
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Transient Synovitis is caused by?
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A viral form of Post-infectious Arthritis, where illness occurs 1-3 weeks earlier
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The differential diagnosis for Juvenille Idiopathic Arthritis includes?
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trauma, malignancy, infection, mechanical, haemarthrosis, paediatric pain syndrome, autoimmune disease
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What clinical presentation would make one think of malignancy as the cause of arthritis?
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fever, weight loss, pain > physical findings, night pain, hepatosplenomegaly, lymphadenopathy, anaemia, metaphyseal lucencies (X-ray)
|
|
Avascular necrosis of the hip is also known as?
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Legg-Perthe's disease
|
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What is the most common cause of hip pain in children age 2-6?
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Transient Synovitis (following viral infection)
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What are the long-term consequences of Transient Synovitis?
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None, the course is benign
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What is the most common cause of hip pain in children age 6-10?
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Legg-Perthe's disease
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what is the most common cause of hip pain in children age 10-14?
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Slipped Epiphysis (a surgical emergency!)
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what autoimmune diseases are associated with arthritis?
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SLE, vasculitities, dermatomyositis, scleroderma
|
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What symptoms are characteristic of growing pains?
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age 3-10, bilateral, usually calf/shin/thigh, worse at night, relieved by massage, able to bear weight, normal labs
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The differential diagnosis for a child that refuses to bear weight, due to pain, is?
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Septic arthritis/osteomyelitis, malignancy, reactive arthritis, pain syndromes, conversion syndrome, reactive sympathetic dystrophy
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Differential diagnosis for a child with night pain?
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Growing pains, Malignancy, Osteoid osteoma
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Still's Disease is eponymous for what form of JIA?
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Systemic JIA
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What is the diagnosis of a boy/girl that presents with arthritis, and a fever & rash that fluctuate daily?
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Systemic JIA (Still's disease)
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What are the complications of Still's disease?
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Hepatosplenomegaly, Lymphadenopathy, Serositis, Pleural effusions, Anaemia, raised Platelets
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what is the stereotypical presentation of oligoarticular JIA?
|
Girl < 5 yrs, swollen large joints, RF-negative, ANA-positive
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what are the complications of oligoarticular JIA?
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knee flexion contracture, quadricep atrophy, leg length discrepancy
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does a negative ANA investigation rule out oligoarticular JIA?
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no, ANA is only 80% sensitive
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what complication of oligoarthritis is associated with positive ANA?
|
uveitis
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what complication of oligoarthritis is asymptomatic, so requires screening exams?
|
uveitis
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what percent of RF negative polyarticular JIA is ANA positive?
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50% (therefore, screen for uveitis)
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what lab investigations are typical of a boy with spondylarthropathy?
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HLA-B27 positive; ANA & RF negative
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what ocular complications come with seronegative spondylarthropathies?
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iritis & conjunctivitis
|
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what alimentary complications occur with seronegative spondylarthropathies?
|
mouth ulcers & IBD
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what percent of JIA will remit within 10 years?
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40%
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what percent of JIA will remit within 5 years?
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30%
|
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what kind of vasculitis is HSP?
|
small vessel
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what acute complication of HSP can lead to death?
|
GI bleeding; intussusception
|
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what chronic complication of HSP can lead to death?
|
ESRD
|
|
what organs are affected in HSP?
|
skin (rash); joints (arthralgia); kidneys (ARF); GIT (bleeds/intussusception)
|
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The diagnositic criteria for HSP is?
|
2 of 4 criteria: a. Palpable purpura, b. Age < 20, at onset, c. Bowel angina, d. Granulocytes in walls of arterioles/venules on biopsy
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|
the differential diagnosis for HSP is?
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acute abdomen, acute scrotum, meningococcal disease, other vasculitis
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basic management of HSP?
|
NSAIDs for symptoms; steroids if abdo pain severe; monitor urine/BP
|
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what is the recurrence rate of HSP?
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1/3
|
|
night sweats, fatigue & malar rash are indicative of a diagnosis of?
|
Juvenile SLE
|
|
what is the major long term compliation of Juvenile SLE?
|
Renal failure
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what percent of SLE cases have onset before age 18?
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20%
|
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stereotypical presentation of dermatomyositis?
|
rash/swelling around eyes, with proximal muscle weakness
|
|
what investigations should be undertaken for dermatomyositis?
|
blood for muscle enzymes; EMG; muscle biopsy
|
|
how is dermatomyositis treated?
|
high dose corticosteroids, tapered down over 18 months. Immunosuppressive agents in relapsing cases
|
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what is the most common complication of dermatomyositis?
|
muscle contractures
|
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morphea' are the characteristic patches of depigmented/hyperpigmented tethered skin of what childhood disorder?
|
Localized Scleroderma
|
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what is the most effective agent for treating localized scleroderma?
|
nothing, at the moment; poor evidence for immunosuppressives
|
|
milestone (hearing): responds to bell.
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2 months
|
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milestone (hearing): turns to bell.
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4 months
|
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milestone (hearing): isolates stimulus above/below head
|
9 months
|
|
milestone (fine motor): grasp cube
|
5 months
|
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milestone (fine motor): transfer cube between hands
|
6 months
|
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milestones (fine motor): bangs cubes together
|
12 months
|
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milestone (fine motor): palmar grasp
|
5 months
|
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milestone (fine motor): pincer grip
|
9 months
|
|
milestone (fine motor): raisin, thumb-finger grasp
|
11 months
|
|
milestone (fine motor): raisin, neat pincer
|
15 months
|
|
milestone (fine motor): crayon, scribbles
|
18 months
|
|
milestone (fine motor): crayon, copies line
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2 years
|
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milestone (fine motor): crayon, circles
|
3 years
|
|
milestone (fine motor): crayon, cross
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4 years
|
|
milestone (gross motor): prone, raises head momentarily
|
1 month
|
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milestone (gross motor): crawls
|
9 months
|
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milestone (gross motor): prone, lifts chest on extended arms
|
6 months
|
|
milestone (gross motor): runs well
|
2 years
|
|
milestone (gross motor): can kick ball without overbalancing
|
2 years
|
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milestone (gross motor): rides tricycle
|
3 years
|
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milestone (gross motor): rolls over
|
4-5 months
|
|
milestone (gross motor): sits unaided
|
9 months
|
|
milestone (gross motor): stands, with support
|
9 months
|
|
milestone (gross motor): walks
|
12 months
|
|
milestone (gross motor): goes up steps
|
2 years
|
|
milestone (gross motor): jumps with both feet together
|
18 months
|
|
milestone (gross motor): stands on one foot
|
3 years
|
|
milestone (gross motor): hopping on one foot
|
4 years
|
|
milestone (gross motor): skipping
|
5 years
|
|
milestone (language): vocalizing
|
2-3 months
|
|
milestone (language): squeals in delight
|
3 months
|
|
milestone (language): laughs
|
4 months
|
|
milestone (language): babbling
|
5 months
|
|
milestone (language): 2-3 words, with meaning
|
12 months
|
|
milestone (language): 5-20 words (recognizes many more)
|
18 months
|
|
milestone (language): 2-3 word sentences
|
2 years
|
|
milestone (language): knows plurals
|
3 years
|
|
milestone (language): asks 'wh' questions; tells fanciful stories
|
4 years
|
|
milestone (language): knows colours
|
4 years
|
|
milestone (language): fluent speech, good articulation
|
5 years
|
|
milestone (social): smiles
|
2 months
|
|
milestone (social): displays pleasure (eg. With bath)
|
3 months
|
|
mileston (social): smiles at self in mirror
|
5 months
|
|
milestone (social): plays peekaboo
|
9 months
|
|
milestone (social): fear of strangers
|
6 months
|
|
milestone (social): responds to own name
|
12 months
|
|
milestone (social): drinks from cup
|
12 months
|
|
milestone (social): indicates toilet needs
|
18 months
|
|
milestone (social): use fork & spoon competently
|
3 years
|
|
milestone (social): gives first name
|
2 years
|
|
milestone (social): knows/names 4 basic colours
|
4 years
|
|
milestone (social): begins fantasy play
|
2 years
|
|
milestone (social): dresses with assistance
|
5 years
|
|
milestone (primitive reflex gone): rooting, sucking
|
4 months (awake); 6 months (asleep)
|
|
milestone (primitive reflex gone): palmar grasp
|
3 months
|
|
milestone (primitive reflex gone): placing and stepping
|
6 weeks
|
|
milestone (primitive reflex gone): moro
|
4 months
|
|
milestone (primitive reflex gone): active tonic neck reflex (ATNR)
|
6 months
|
|
T or F: Gastroenteritis can present as vomiting without diarrhea?
|
False, vomiting alone does NOT equal gastroenteritis
|
|
What is Ramsay-Hunt syndrome?
|
when shingles appear in CN VII and causes palsy
|
|
what is a pregnant women treated with, if she contracts chickenpox?
|
VZIG
|
|
a flat, impalpable rash is called?
|
a macule
|
|
an elevated rash (with no fluid or pus) is called?
|
a papule
|
|
if a child is T-cell deficient, where are the common sites of spread for Chickenpox?
|
Lungs and Liver
|
|
how is chickenpox usually diagnosed?
|
Clinically
|
|
how is chickenpox treated?
|
oral antivirals (high dose); IV in severe cases
|
|
what is the vaccine for chickenpox?
|
VZV Oka (live attenuated); booster at 10yrs
|
|
how effective is the Oka vaccine?
|
Overall 80-85%; from severe disease 95%
|
|
What is Herpetic Whitlow?
|
HSV infection of the digits, which normally lasts 2-3wks (1/5 will recur)
|
|
when is the worst time in pregnancy for a mother to contract genital herpes?
|
late in pregnancy
|
|
what is the mortality rate for neonatal HSV infection? What is the treatment?
|
Mortality = 25%. Treatment = IV acyclovir
|
|
what percent of RTI are viral? Bacterial?
|
viral: 90%; bacterial 10%
|
|
what are the two most common causes of cough in a child?
|
a) asthma, b) viral bronchitis
|
|
what is the definition of bronchiolitis?
|
first presentation of wheezing, URTI and respiratory distress
|
|
when is the peak incidence of bronchiolitis?
|
at 6mo (<2 yrs), in the winter/early spring
|
|
bronchiolitis in childhood is a risk factor for which disease later on?
|
asthma (hyper-reactive airways)
|
|
over 50% of bronchiolitis is caused by RSV. What are other viruses commonly responsible?
|
parainfluenzae, influenzae, rhinovirus, adenovirus
|
|
what is the presentation of bronchiolitis (including prodrome)?
|
[prodrome] of URTI w/cough & fever; [5-6days] irritable, poor feeding, wheeze, respiratory distress, tachycardia, retractions, poor air entry
|
|
what investigations need be done for bronchiolitis?
|
a) CXR, b) NP swab, c) FBC - usually WBCs normal
|
|
how is bronchiolitis managed?
|
supportive (fluids, antipyretics, O2sat>92%) +/- ventolin [hospitalize if severe]
|
|
what would define bronchiolitis to be severe, and in need of hospitalization?
|
RR > 60; O2sat < 92%; age < 6m; feeding issues; prior hospitalization for bronchiolitis
|
|
when are children at highest risk of pneumonia?
|
in 1st year of life
|
|
what is the difference in clinical presentation of viral and bacterial pneumonia?
|
[viral] cough, wheeze, stridor; [bacterial cough, fever/chills, dyspnoea
|
|
what is the difference on CXR, for viral vs bacterial pneumonia?
|
[viral] diffuse, streaky; [bacterial] lobular, consolidation w/possible pleural effusion]
|
|
what is the treatment of pneumonia?
|
supportive: O2, hydration, antipyretics
|
|
what is the technical name for Croup?
|
subglottal laryngitis?
|
|
peak incidence of Croup?
|
ages 4m to 6y (in fall or early winter)
|
|
what are the leading causative organisms for Croup?
|
parainfluenzae (75%), influenzae A/B, RSV, adenovirus
|
|
what is the clinical presenation of Croup?
|
hoarse voice, stridor, barking cough, worsening symptoms at night
|
|
a child is presented for a 'barking' cough, which has been waking them at night, and you observe their voice is harsh and stridor is heard on exam. What is your clinical diagnosis?
|
Croup
|
|
how is Croup managed?
|
O2 + steroids (1 dose, dexa) +/- nebulized adrenalin +/- intubation [if severe]
|
|
common name for Pertussis?
|
Whooping cough (or 100-day cough)
|
|
Medical name for Whooping Cough?
|
Pertussis
|
|
Causative organism in Pertussis?
|
Bordetella pertussis
|
|
When is someone with Pertussis infectious?
|
1 week prior to onset; 3 weeks after
|
|
Is Whooping Cough contageous?
|
Highly so!
|
|
What age group typically gets Pertussis?
|
Bimodal distribution: <1 yr & adolescents
|
|
How does Pertussis present (including prodrome)?
|
[prodrome] (1-2w) of catarrhal, coryza, mild cough; [paroxysmal] (2-4w) paroxysmal cough followed by whoop (absent in adults/some infants) +/- vomiting, apnoea
|
|
How long does Pertussis last?
|
General symptoms: 6 weeks. Cough: up to 6 months
|
|
Complications of Pertussis?
|
subconjunctival haemorrhage; rectal prolapse; hernias; epistaxis
|
|
When is Whooping cough most infectious? Least?
|
Most - prodromal period; Least - Convalescent period
|
|
How is Pertussis diagnosed?
|
Clinically, based on URTI followed by paroxysmal cough, in an afebrile pt
|
|
What is the expected temperature of a patient with Pertussis?
|
Afebrile
|
|
How is the diagnosis of Whooping Cough confirmed?
|
PCR of NP swab
|
|
Treatment for Pertussis?
|
Supportive + Erythromycin (40mg/kg PO x10d)
|
|
When should pertussis pts be hospitalized?
|
When paroxysms results in apnea or cyanosis
|
|
what is the purpose of erythromycin for whooping cough?
|
NOT to decrease course, but to reduce infectivity
|
|
how long must a pt w/pertussis be isolated?
|
until 5 days after treatment of erythromycin has commenced
|
|
what pertussis vaccine is available for kids?
|
Pentacel (acellular)
|
|
what pertussis booster is given to adolescent/adults?
|
Adacel
|
|
what viruses are responsible for Hand Foot and Mouth disease?
|
enteroviruses (mainly Coxsackie virus 16)
|
|
which enterovirus is associated with neurological demyelination/encephalitis, in Hand, Foot and Mouth Disease?
|
EV17
|
|
How does Hand Foot and Mouth disease present?
|
Papular-Vesicular eruption of the mouth/hands/feet, and sometimes buttocks
|
|
where in the mouth are the lesions with HFM disease?
|
posteriorly; gums are not involved
|
|
Vesicular rash around the mouth, which develop a honey-coloured crust is characteristic of what infection? What are the causative organisms?
|
Impetigo, caused by Staph or Strep
|
|
What rash is preceded by acute catarrhal illness, fever and Koplik spots?
|
Measles
|
|
how is measles diagnosis confirmed?
|
PCR or IgM detection (3d after rash starts)
|
|
what are the serious complications of measles?
|
respiratory; nervous system
|
|
describe the rash of measles
|
maculopapular, blotchy, red/pink; starts behind ears, moving downwards; becomes confluent on upper body
|
|
how does measles progress, after the rash appears?
|
skin will turn brown; rash fades after 2-3d; desquamation occurs (not hands/feet, though)
|
|
how is measles treated?
|
Supportive therapy
|
|
what are Koplik's spots?
|
(unique to measles) small red spots with bluish/white centres, occuring in the mucous membrane of the mouth
|
|
what is the other name for Rubella?
|
German Measles
|
|
How does Rubella present?
|
rash (usually first sign) which descends from face; lesions remain discrete; LAD (posterior/cervical); shorter course than measles; desquamation not characteristic
|
|
what organism causes Scarlet Fever?
|
Group A Streptococci
|
|
A rash that spares the region around the mouth, but affects the neck/skin folds & has a 'strawberry tongue' is likely?
|
Scarlet Fever
|
|
how does Scarlet fever present?
|
Strawberry tongue', with a rash that is most prominent on neck & skin folds (sparing around mouth); desquamation of face>trunk>limbs
|
|
how is Scarlet fever managed?
|
10 days (min) Abx, to prevent long term sequelae (such as Acute Rheumatic Fever, Glomerulonephritis)
|
|
what is the cause of Kawasaki Disease?
|
The cause is presently unknown
|
|
how does Kawasaki Disease present?
|
fever (5d), plus 4 out of 5: i) bilateral conjuntival injection, ii) rash, iii) red mouth/tongue/throat & cracked lips, iv) hands/feet: swelling > desquamation, v) cervical LAD
|
|
what is the major complication associated with Kawasaki Disease?
|
Coronary Artery Aneursyms
|
|
How is Kawasaki Disease treated?
|
ASA & IVIG
|
|
What organisms causes Erythema infectiosum (aka Fifth Disease)?
|
Parvovirus B19
|
|
How does Erythema infectiosum present?
|
mild fever & rash; rash starts with 'slapped cheek' -> lacy, maculopapular over 1-2 weeks; arthralgia
|
|
how is erythema infectiosum diagnosed?
|
PCR (blood), or parvovirus IgM
|
|
how is erythema infectiosum treated?
|
supportive
|
|
what causes Roseola Infantum?
|
HHV-6 (Human Herpes Virus 6)
|
|
presentation of Roseola Infantum?
|
high fever (3-4d), which falls with rash onset; rash is maculopapular, mainly trunk; child looks WELL
|
|
a rash in a well-looking child, which is preceded by several days of high fever is characteristic of?
|
Roseola Infantum
|
|
How is meningococcal infection diagnosed?
|
Rash, Gm (-) from blood/CSF/petechiae, PCR of blood/CSF
|
|
what iare the major complications associated with Meningococcal disease?
|
DIC and thrombocytopenia (hence petechial rash); meningitis; septicaemia
|
|
how is meningococcal infection managed?
|
IM penicillin (community); 3rd G cephalosporin (hospital); antibiotic prophylaxis to close contacts
|
|
what is the serotype of swine flu? Avian flu?
|
swing: H1N1; avian: H5N1
|
|
what extrapulmonary site is commonly infected in childhood TB?
|
CNS (but also, bone, renal, GIT)
|
|
what antibiotic for TB is avoided in children?
|
ethambutol (not < 5yrs age)
|
|
how is TB diagnosed?
|
Quantiferon (IFN-gamma); TST/Mantoux; culture; PCR
|
|
what gene is associted with MRSA?
|
Panton-Valentine Leucocidin gene
|
|
what antibiotics should be considered for MRSA?
|
erythromycin (and clindamycin or linomycin) if sensitive; if not, vancomycin
|
|
what workup should be done for any infant with a fever?
|
culture: blood, CSF, urine, NP swab, focal lesions (start on empirical antibiotics)
|
|
what are the common culprits for bacterial meningitis in a neonate?
|
GBS & E coli (minor: GNR, L monocytogenes, Strep pneumoniae, Enterococci, N meningitis, S aureus)
|
|
how does neonatal sepsis present?
|
[early; <72hr] fulminant mulisystem disease with pneumonia; [late] focal infections, meningitis
|
|
in neonatal sepsis, what pathogens might cause brain abscesses?
|
Enterobacter sakazakii & Citrobacter spp
|
|
what is the most common congenital infection in Australia?
|
congenital CMV
|
|
90% of congenital CMV is asymptomatic, but how do the other 10% present at birth?
|
microcephaly/IUGR; jaundice; petechiae; chorioretinitis; heptosplenomegaly
|
|
what is the triad of abnormalities noted with congenital rubella?
|
eyes (cataracts), cardiac, CNS (deaf, microcephalic)
|
|
what is a late manifestation of congenital rubella?
|
Insulin dependant DM
|
|
what are the four organisms associated with congenital eye infections?
|
N gonorrheae, Chlamydia, S aureus, Pseudomonas aeruginosa
|
|
What fluids are provided for resuscitation?
|
bolus: 20ml NS/kg (no glucose) then reassess. Repeat, up to 40ml/kg [total]
|
|
what is the formula for calculating fluid deficit?
|
% dehydration x 10 x wt(/kg) [eg. 5% x10 x 22kg = 1100ml/24hr]
|
|
how often should fluid status/electrolytes be reassessed in a child receiving fluids?
|
at least every 24 hours, but q4-6h if unwell
|
|
how are maintenance fluids calculated?
|
100ml/kg [first 10kg] + 50ml/kg [10-20kg] + 20ml/kg [>20kg] (eg. 22kg child will receive 1540ml/d)
|
|
what fluids are used for fluid deficit and maintenance?
|
0.9% NaCl + 5% glucose
|
|
how much potassium is added to fluids, if hypokalaemia is noted?
|
20mmol/L of NS
|
|
for patient controlled analgesia, what does of morphine is added to continuous IV saline?
|
10mcg/kg/hr
|
|
if receiving PCA, what is the hourly dose limit for morphine?
|
150mcg/kg
|
|
what is the prefered antiemetic for children, and what is the dose?
|
ondansetron (0.1mg/kg)
|
|
if a dehydrated child is noted to be hypernatraemic, how is rehydration modified?
|
fluid deficit should be calculated as normal (% x10 xkg), but administered over 48hrs instead [NS still!]
|
|
if using ORT therapy, instead of IV, what rate should the child be drinking?
|
5ml q5min
|
|
what is the incidence of cystic fibrosis?
|
1:2500 births (Caucasians)
|
|
what is the most common presentation of CF, at birth?
|
meconium ileus & bilious vomiting
|
|
why do CF patients suffer chronic diarrhoea?
|
due to pancreatic insufficiency
|
|
what dietary considerations are necessary for CF patients?
|
high caloric intake (poor absorption); vitamins A, D, E, K (fat soluble)
|
|
what is acholic stool an indicator of in an infant?
|
biliary atresia, or cirrhosis
|
|
what is the incidence of coeliac disease?
|
1 in 100
|
|
what is the cause of Coeliac disease?
|
immune-mediated sensitivity to gluten
|
|
how does Coeliac disease present in an infant?
|
FTT, diarrhoea, abdominal pain, vomiting, constipation, abdominal distension, irritability
|
|
how does one screen for Coeliac disease?
|
screen for EMA (endomysial antibody) & IgA to TTG (tissue transglutaminase protein), then biopsy of bowel
|
|
what are the indications for screening for Coeliac disease in a neonate?
|
iron deficiency anaemia, IgA deficiency, Down/Turner/Williams syndrome, first-degree relative with CD
|
|
what is the most sensitive and specific means for detecting Coeliac Disease?
|
IgA antibody to TTG
|
|
how is Coeliac Disease managed?
|
Gluten-free diet (GFD) for life, with monitoring of TTG (for adherence/response)
|
|
how often should Coeliac's have their TTG measured?
|
6 months after diagnosis, then annually thereafter
|
|
how does one assess watery stools in an infant?
|
use Clinitest to detect if Reducing Substances are >0.5%
|
|
if diarrhoea is found to be positive for reducing substances, what further investigation should be undergone?
|
biopsy small bowel
|
|
why is determining reducing substances in stool difficult in the community?
|
the sample must be fresh, otherwise bacterial fermentation may result in a false reading
|
|
what is the formula for determing the osmotic gap in stool fluid?
|
280 (serum osmolality) - 2[Na+K] = osmotic gap
|
|
what are the likely causes of watery stool, if it is negative for reducing substances?
|
secretagogue (VIP), congenital transport defect, infection, malabsorbed bile salts
|
|
what does the enzyme sucrase break sucrose down in to?
|
glucose and fructose
|
|
what does the enzyme lactase break lactose down in to?
|
glucose and galactose
|
|
how is fructose transported into enterocytes?
|
by GLUT-5 (non-energy dependant)
|
|
how are glucose and galactose absorbed by enterocytes?
|
by SGLT1 cotransporter (energy dependant)
|
|
osmotic diarrhoea occurs when the osmotic gap is …?
|
> 100
|
|
what dietary sweetener may act as a laxative?
|
sorbitol
|
|
how is diarrhoea due to malabsorption treated?
|
remove the offending sugar from the diet permenantly
|
|
why are perianal excoriation and hyperchloraemic acidosis associated with osmotic diarrhoea?
|
1) unabsorbed carbs are fermented to short-chain fatty acids in the large bowel [excoriation], 2) and these are absorbed in exchange for bicarbonate [acidosis]
|
|
osmotic diarrhoea upon introduction of fruit into the diet is indicative of what?
|
Congenital Sucrase-Isomaltase Deficiency
|
|
which is more common: mono- or disaccharide deficiency?
|
disaccharide deficiency, by far
|
|
what are some causes of disaccharide malabsorption?
|
viral gastro, Coeliac disease, Chronic giardiasis, milk protein enteropathy, small bowel bacterial overgrowth syndrome, immunodeficiency disorders, autoimmune enteropathy
|
|
how is the recessively-inherited monosaccharide malabsorption treated?
|
substitution of fructose for glucose-galactose
|
|
what osmotic gap is seen in secretory diarrhoea?
|
osmotic gap < 50
|
|
is secretory diarrhoea usually congenital or acquired?
|
acquired, by far
|
|
what is the differential diagnosis for bloody diarrhoea in a child?
|
infection (bacterial, parasitic) or IBD (Crohn's, UC, milk protein intolerance)
|
|
findings of leukocytes in the stool is indicative of?
|
colitis
|
|
how is protein-losing enteropathy assessed?
|
by alpha-1-antitrypsin levels in the stool
|
|
why does blood in the stool sometimes result in iron deficiency and oedema?
|
due to excessive blood and protein loss
|
|
bacteria account for 15% of acute gastroenteritis. Who are the main offenders?
|
Campylobacter jejuni, Salmonella, enteropathogenic E. coli, Shigella, Yersinia enterocolitica, Shiga-toxin producing E. coli, Cholera
|
|
what protozoans cause acute gastroenteritis?
|
cryptosporidium, giardia lamblia, entamoeba histolytica
|
|
what helminth can cause acute gastroenteritis?
|
Strongyloides Stercoralis
|
|
Viruses cause >70% of acute gastroenteritis. Who are the main offenders?
|
rotaviruses, adenoviruses, small round viruses, caliciviruses, astroviruses, enteroviruses
|
|
if a child is thirsty, has reduced urine output, dry mucous membranes and mild tachycardia, what is their level of dehydration?
|
'mild' or 3%
|
|
if a child is thirsty, tachycardic, has an abnormal respiratory pattern & sunken eyes, what is their level of dehydration?
|
'moderate' or 5%
|
|
if a child is thirst, has sunken eyes, poor perfusion & signs of shock, what is their level of dehydration?
|
'severe' or 10%
|
|
capillary refill correlates to degree of dehydration, but can be unreliable in light of …?
|
fever, or high ambient temperature
|
|
what investigations should be undergone for mild dehydration due to diarrhoea?
|
it is not necessary in this case
|
|
what investigations should be undergone for moderate to severe dehydration due to diarrhoea?
|
FBC, UEC*, blood sugar, stool (micro, culture, virology). [consider: urine tests, blood culture if febrile, imaging]
|
|
what percent dehydration is assumed in a child requiring IV fluid therapy?
|
5%
|
|
what is first line therapy for rehydration of a child?
|
oral rehydration therapy (ORT)
|
|
what is in gastrolyte?
|
glucose, sodium, potassium, chloride, citrate
|
|
why is glucose required for ORT?
|
because sodium and glucose are cotransported in an equimolar ratio
|
|
what is the most common cause of gastroenteritis?
|
viral (rotavirus)
|
|
what is required for a diagnosis of gastroenteritis?
|
increase in water stools (often with vomiting and fever)
|
|
what is malabsorption?
|
failure to absorb nutrients
|
|
what is the most common presentation of malabsorption?
|
diarrhoea
|
|
how does fat enter circulation, after diffusion across enterocyte apical membranes?
|
reconstituted by chylomicrons (containing apoB), and enters lymphatic system
|
|
what organs are usually dysfunctional in the case of steatorrhea?
|
pancreas, or small intestine
|
|
if fat globules are found in stool, what investigations should be performed?
|
1) breath test, 2) sweat test [CF], 3) formal 3d faecal fat balance study
|
|
if fat acid crystals are found in stool, what investiations should be performed?
|
small bowel biopsy
|
|
How is the diagnosis of Impetigo confirmed?
|
lesion swabs (C&S)
|
|
How is impetigo managed?
|
C&S, oral antibiotic (7-10d), antiseptic bath oil (skin colonisation), wash clothing/linens, bactroban (nasal colonisation)
|
|
folliculitis is mostly caused by what organisms?
|
S aureus, Dermatophytes (tinea), P aeruginosa
|
|
how is folliculitis S aureus managed?
|
C&S, Abx (7-10d), treat prediposing factors (eg. Dermatitis), bactroban (nasal colonization)
|
|
where is folliculitis dermatophyte usually contracted from?
|
pets (eg. Guinea pig)
|
|
how is folliculitis dermatophyte diagnosed & treated?
|
Dx: scrapings (microscopy/culture); Tx: griseofulvin (8-10wks)
|
|
where is pustular folliculitis Pseudomonas acquired from?
|
heated pools, spas, bath toys
|
|
how is folliculitis Pseudomonas treated?
|
Avoidance of source (will resolve on own)
|
|
what is the medical name for Ringworm?
|
Tinea
|
|
how does tinea present?
|
progressive enlargement of area, usually skin/scalp +/- pruritis; usually unilateral; may cause incomplete alopecia
|
|
how is tinea treated?
|
[localized] topical antifungal for 6wk; [widespread] oral griseofulvin (2-3months); [nails] oral lamisil (3-6months) + loceryl nail lacquer
|
|
what causes molluscum contageosum?
|
Pox virus
|
|
how does molluscum contageosum appear?
|
skin coloured papules which umbilicate +/- eczema
|
|
how long does molluscum contageosum take to resolve?
|
6-12 months
|
|
how is molluscum contageosum managed?
|
showering with no abrasives; diluted imiquimod cream (speeds up); gently prick papules
|
|
what types of lesions may human papillamo virus cause?
|
normal verrucae, plane warts, plantar warts, condyloma accuminata
|
|
how is atopic dermatitis managed?
|
a) topical steroids +/- wet dressings, b) phototherapy, c) topical tacrolimus, d) oral immunosuppression [cyclosporin, azathioprine]
|
|
how are topical steroids used in atopic dermatitis?
|
q12-24hr, 10d on/4d off (or variant) until clear
|
|
what environmental factors trigger atopic dermatitis?
|
irritants*, infections*, food/airborne allergens, food intolerance
|
|
what are five common irritants that exacerbate atopic dermatitis?
|
water, soap, shampoo, chlorine, wool, nylon, acrylic, carpet, sandpits, heat, fragrances, preservatives
|
|
if atopic dermatitis is found to be supercolonized with S aureus, how is it managed?
|
antibacterial bath oil; low dose bactrim
|
|
if food allergies are suspected to be a cause of atopic dermatitis, what investigations can be performed?
|
SPTs/RAST, food challenges (natural chemicals, colouring, preservatives)
|
|
what topical therapy is available for treating psoriasis?
|
steroids, tar, dithranol, daivonex
|
|
is sunlight beneficial or detrimental for psoriasis?
|
beneficial (UVB)
|
|
what oral therapies are available for severe psoriasis?
|
methotrexate, cyclosporin
|
|
what is the most common complication of haemangiomas?
|
ulceration, due to growth which exceeds vascular supply (painful!)
|
|
how are ulcerated haemangiomas treated?
|
oral propanalol
|
|
if a haemangioma is at risk of causing cosmetic or functional problems (ie. On the eye or face) how is it managed?
|
oral/intralesional steroids, or vincristine, or interferon
|
|
haemangiomas in what locations are a clue to underlying problems?
|
mandibular area (larynx), gluteal cleft (urogenital)
|
|
what does PHACE stand for and what is the relation to haemangiomas?
|
[p]osterior cranial fossa, [h]aemangioma, [a]rterial abnormalities, [c]coarctation of aorta, [e]eye anomalies
|
|
what is the GFR of a newborn?
|
about 20
|
|
what is the GFR of a 2 year old?
|
about 130
|
|
what is the normal BP of a 1 year old?
|
[85-105]/[40-60]
|
|
what is the normal BP of a 10 year old?
|
[100-120]/[60-80]
|
|
at what age is a child usually toilet trained?
|
age 2-3
|
|
antenatal U/S will detect renal anomalies in what % of fetuses?
|
1%
|
|
rate of nighttime enuresis in 5 year olds? 10 year olds?
|
5 yrs: 10%. 10 yrs: 5%
|
|
what does MCUG stand for?
|
Micturating CystoUrethroGram
|
|
what is grade II vesico-ureteric reflux (VUR)?
|
contrast is in non-dilated ureter & renal pelvis
|
|
what study would be used to investigate if a child has vesico-ureteric reflux?
|
an MCUG (micturating cystourethrogram), where dye is instilled in bladder, voided, then imaged
|
|
what is grade IV vesico-ureteric reflux?
|
moderately turtuous ureter, & dilatation of the pelvic/calyses
|
|
what form of renal imaging is useful for eliciting scars?
|
DMSA (dimethyl succinate acid), which requires IV radioisotope injection
|
|
what imaging modality is good for determining GFR of the kidneys?
|
DTPA
|
|
what imaging modality is good for determining differential function between kidneys?
|
DMSA, DTPA and MAG3
|
|
what imaging modality is good for determining if a renal/ureteric obstruction is present?
|
DTPA or MAG3
|
|
what percent of febrile children have a UTI?
|
5%
|
|
what percent of girls will get a UTI before age 7?
|
8%
|
|
what is the recurrence rate of UTIs in children?
|
1 in 3
|
|
the most common agents causing UTI in children is?
|
E coli (>80%) [followed by Klebsiella, at 10-15%]
|
|
what percent of children with a UTIl also have VUR?
|
30%
|
|
symptoms of a UTI in an infant?
|
poor feeding, PUO, vomiting, 'not right', jaundice
|
|
symptoms of a UTI in a young child?
|
dysuria, abdo/loin pain, wetting, haematuria, fever
|
|
how should one obtain urine from an child who has a UTI?
|
bag urine, clean catch, in-out catheter, suprapubic tap
|
|
for a well child (febrile or afebrile), what is the treatment for a UTI?
|
cephalexin or bactrim PO [afebrile: 3d, febrile 7d]
|
|
for a neonate or an unwell child with a UTI, what is the treatment?
|
ampicillin & gentamicin IV [until appropriate to switch to oral]
|
|
lifestyle modifications to prevent UTIs?
|
regular fluids/voiding, treat constipation, avoid caffeine, correct wiping, cranberry supplements
|
|
how is known VUR treated?
|
circumcision [boys], prophylactic antibiotics, surgery [deflux]
|
|
on first presentation of a typical UTI in an infant, what imaging should be performed?
|
ultrasound [6 weeks after infection]
|
|
for recurrent UTI, what imaging should be performed?
|
Ultrasound [during infection], DMSA [4-6m after], MCUG
|
|
on first presentation of a typical UTI in children >6 months, what imaging should be performed?
|
None (only U/S if <6mo)
|
|
what imaging should be done for a child with recurrent UTIs, if older than 6mo?
|
U/S [within 6w of infection], consider DMSA
|
|
2% of UTIs are asymptomatic. How are they treated?
|
No treatment is required
|
|
how does oedema differ between nephrotic and nephritic syndrome?
|
nephrotic: severe, insidious. Nephritic: milder, but sudden onset
|
|
where would one find hypertension: nephrotic or nephritic syndrome?
|
often found in nephritic syndrome (not in nephrotic)
|
|
what form of GN would one find raised creatinine?
|
often found in nephritic syndrome (not in nephrotic)
|
|
what is the triad of symptoms in nephrotic syndrome?
|
oedema, proteinuria, hypoalbuminaemia
|
|
how is a first episode of nephrotic syndrome treated?
|
prednisone (effective in 80%). PO 60mcg/m2 x 4wks, then 40mcg/m2 q2d x 4wks, weaning over 1-5 months
|
|
what is the most common form of nephrotic syndrome in children?
|
MCNS (minimal change nephrotic syndrome)
|
|
what age range is MCNS common in?
|
1-10 yrs
|
|
aside from steroids, how is nephrotic syndrome managed?
|
teach parents urine dipstick; fluid restrict until remission; no added salt; pneumococcal vaccine
|
|
what vaccine should be given to children with nephrotic syndrome?
|
pneumococcal
|
|
complications of nephrotic syndrome?
|
infection, especially encapsulated [loss of IgG, complements, T-cell dysfxn], thromboemboli [loss of clotting factors, coag inhibitors], cardiovascular, drug side effects
|
|
how does one manage acute nephritis if PI/PSGN?
|
none necessary
|
|
how is acute nephritis managed?
|
diuretics [oedema], antihypertensives, treat ARF, immunosuppresants
|
|
how is ARF managed?
|
fluid/electrolyte correction, correct HTN, remove toxins, dialyze (if necessary)
|
|
what form of G/N has a poor prognosis?
|
FSGS
|
|
what are common causes of CRF in children?
|
renal dysplasia, obstructive uropathy, G/N, reflux nephropathy
|
|
what is the definition of hypertension in a child?
|
SBP or DBP >95C
|
|
what is the normal resp rate for an infant <1yr? HR?
|
(RR) 30-40bpm. (HR) 110-160bpm.
|
|
what is the normal resp rate for a child between 5-12? HR?
|
(RR) 20-25. (HR) 80-120
|
|
name ED red flags for children?
|
purpuric rash; bulging fontanelle; biphasic stridor; high pitched scream; bile stained vomit; persistent tachycardia; grunting respiration
|
|
signs of respiratory distress?
|
recession; grunting; nasal flaring; tachypnoea; accessory muscles; head bobbing; cyanosis
|
|
what are the ABCDs to assess/monitor in ED?
|
Airway, Breathing, Circulation, Disability/dehydration
|
|
what is the normal capillary refill time (CRT) for a child?
|
CRT < 2s
|
|
what is considered 'reduced fluid intake'?
|
less than 1/2 of normal daily intake
|
|
what is considred 'reduced urine output' in an infant?
|
<4 wet nappies in 24hrs
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how are arms positioned in decorticate posturing? Decerebrate?
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Decorticate: elbows flexed. Decerebrate: elbows extended; pronated
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what are indications for intubation, in severe bronchiolitis?
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PaO2 < 50; rising PaCO2; fatigue; inability to protect airway; severe metabolic acidosis; depressed LOC; recurrent apnoea
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what is the major feature of severe asthma?
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pulsus paradoxus!
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a febrile infant is considered 'toxic' when?
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lethargic; poor feeding; irritable; poorly perfused; reduced urine output
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what sites of infection should one think of in a febrile child?
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meningitis; pneumonia; UTI; otitis media; tonsillitis
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what is the most common cause of a febrile episode in children?
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viral infection (>90%)
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what investigations are part of a 'septic workup' in a febrile child?
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FBC/film, cultures: blood/stool, CXR, urinalysis, LP
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how are fits/seizures managed in a child?
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[during] airway protection (lie on side), [after] semi-prone position, paracetamol
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if a seizure is persistent, how is it managed?
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check BSLs; diazepam (0.2mg/kg IV or 0.5mg/kg PR); phenytoin 20mg/kg; consider thiopentone (muscle relaxation)
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what vaccines are given at birth?
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HepB
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what vaccines are given at 12 months?
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MMR, MeningC, Hib (4th)
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what vaccine is given at 18 months?
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VZV (Oka)
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when is DTPa immunization given?
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2/12, 4/12, 6/12, 3.5yrs, 15yrs, 50yrs
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what vaccine preventable disease is on the rise in NSW?
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pertussis (mutation?)
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what is the concentration of adrenalin used in an anaphylactic reaction?
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1 in 1000
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at what age does one administer vaccines into the deltoid?
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>12 months
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what manoeuvre is classically used for Duschenne Muscular Dystrophy?
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Gowers' manoeuvre (standing from a seated position: splits legs, and pushes self up with arms)
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what finding might one expect in the legs of a child with Muscular Dystrophy?
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Calf hypertrophy
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what finding is common on muscle biopsy of neuropathic origin?
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'grouped' atrophy of fibres
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how does Spinal Muscular Atrophy (SMA) type 1 present?
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marked proximal weakness, decreased movement, bell-shaped chest, mobile/expressive face, normal intelligence, tongue fasciculations, absent reflexes
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how does one screen SMA if it is suspected?
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genetic testing: SMN gene
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what are the presenting features of Charcot Marie Tooth disease?
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Pes cavus (feet); 'clawed' hands
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why can't the sural nerve be biopsied for diagnosis of CMT disease?
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as it is a sensory nerve, and Charcot Marie Tooth is a (peripheral) motor neuron disease
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what is the most commonly inherited muscle disease?
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Duschenne Muscular Dystrophy, 1 in 3500 boys (X-linked)
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what is the primary cause of death in DMD?
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cardiomyopathy or respiratory insufficiency
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what is the average lifespan for children with DMD?
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teens-twenties
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is IQ normal in DMD?
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no, it is 10 points lower, on average
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how is muscular dystrophy diagnosed?
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[1]blood: CK; [2]muscle biopsy: histology, biochem, western blot (dystrophin absent!), mutation analysis
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how does Becker Muscular Dystrophy differ from DMD?
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milder phenotype; in-frame deletion; same pattern of weakness
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what protein is absent on WB of a muscle biopsy in a DMD patient?
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dystrophin
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what are the cornerstones of extending a DMD patient's life expectancy?
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1) corticosteroids, 2) spinal surgery, 3) ventilation
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what medication should be considered for muscular dystrophy?
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intermittent steroids (+bisphosphonates)
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at what age is the 'plateau phase' of muscular dystrophy?
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4-5 years
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what experimental treatment for muscular dystrophy is still pending phase II results?
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weekly IM injections of PTC124, which skips premature stop-codon
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