GENERAL PEDIATRICS M Flashcards

1
Q

Signs and symptoms of cocaine use?

A
Euphoria
Dilated pupils
Increased motor activity
Tachycardia
Hyperthermia
Hypertension 
May have nasal mucosal changes
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2
Q

Signs and symptoms of PCP (phencyclidine, aka “angel dust”, “wet”, “illy”) use?

A
Nystagmus 
Ataxia
Hypersalivation
Emotional lability
Altered sensory perception and hallucinations about body image leading to panic and disorientation
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3
Q

What are the two most commonly used drugs in adolescents?

A
  1. Cigarettes
  2. Alcohol

Most common illicit drug: marijuana

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4
Q

What are clinical features of Trisomy 18 (ie. Edwards Syndrome)?

A
Rocker bottom feet
Microcephaly
Inguinal hernias
Low set, small ears
Congenital cardiac defects
Clenched hands with overlapping digits
Cleft lip and palate
Micrognathia
Shield chest
Prominent occiput
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5
Q

What are clinical features of Trisomy 13 (ie. Patau Syndrome)?

A
SGA
Cutis aplasia (skin missing from scalp)
Microcephaly
Polydactyly
Clenched hands with overlapping digits
Microophthalmia
Omphalocele
Holoprosencephaly
Cleft lip and palate
Congenital heart defects
Rocker bottom feet
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6
Q

What is the most common inherited cause of mental disability?

A

Fragile X syndrome: 1 in 2000 live births

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7
Q

What screening bloodwork can be ordered in a child with FTT and normal vitals and physical examination?

A
  1. CBC - screen for anemia secondary to inadequate nutritional intake
  2. Urinalysis and urine culture - screen for renal tubular acidosis
  3. Serum electrolytes, BUN, Cr
  4. Gas - screen for renal tubular acidosis
    Then tailor the rest of the investigations based on history and physical exam findings
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8
Q

What is the underlying cause of Fragile X?

A

Expansion of the familial mental retardation gene (FMR1) on the long arm of the X chromosome at q27.3
-once trinucleotide repeat > 200, get Fragile X!

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9
Q

What are clinical features of Fragile X syndrome?

A
Macrocephaly
Macroorchidism after puberty
High arched palate
Large ears
Can have autism!!!
Joint laxity
Dental crowding
Mild to severe mental disability
ADHD
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10
Q

What are babies on a goat’s milk diet susceptible to?

A
  1. Megaloblastic anemia (secondary to folate and vitamin B12 deficiency)
  2. Iron deficiency anemia
  3. Brucellosis
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11
Q

What are the clinical features of Kasabach-Merritt Syndrome?

A
  1. Large hemangioma (vascular tumor) anywhere in body
  2. Thrombocytopenia
  3. Consumptive coagulopathy
  4. Hemolytic anemia (secondary to microangiopathic destruction)
  5. Risk of bleeding
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12
Q

What are the two major differential diagnoses for cafe-au-lait spots?

A
  1. Neurofibromatosis type 1

2. McCune Albright syndrome

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13
Q

What are the clinical features of McCune-Albright Syndrome?

A

THINK BONE AND HYPER-ENDOCRINOPATHY!

  1. Polyostic fibrous dysplasia
  2. Cafe au lait with jagged edge “Coast of Maine” look
  3. Hyperthyroidism
  4. Precocious puberty
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14
Q

What are the main features of tinea capitis? (5)

A
  1. Scaly alopecia
  2. Blackdots hairs
  3. Posterior cervical adenopathy
  4. Most common cause: Trichophyton tonsurans
  5. Potassium hydroxide test often positive
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15
Q

What is the mode of inheritance of Marfan Syndrome?

A

Autosomal dominant

De novo mutation in 1/4 of Marfan’s Syndrome

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16
Q

What is the underlying defect in Marfan syndrome?

A

Defect in fibrillin protein

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17
Q

What type of growth pattern occurs in Marfan Syndrome? (4)

A
  1. Increased linear long bone growth
  2. Taller than predicted based on mid-parental height
  3. Increased growth velocity, occurs 2 years earlier than expected
  4. Decreased muscle mass (weight < 50th percentile)
18
Q

Why is gross motor delay common in Marfan Syndrome patients?

A

Joint laxity

19
Q

Which body systems are affected in Marfan Syndrome? (6)

A
  1. Ocular
  2. Cardiac
  3. Respiratory
  4. GI
  5. MSK
  6. Derm

**No neurological effects

20
Q

What ocular issues occur in Marfan Syndrome?

  • most common?
  • 5 total
A

Most common: 1. myopia (progressive, early onset)

  1. Early cataracts
  2. Glaucoma
  3. Superior ectopia lantis (60% of cases): lens displacement up and out (tell-tale sign of Marfan’s)
  4. Retinal detachment
21
Q

What face and mouth findings do you see in Marfan Syndrome?

A
Elongated and narrow face
Micrognathia
High arched palate
Dental crowding
Inophthalmos (deep set eyes and downward slanting)
Malar hypoplasia
22
Q

What are possible cardiac abnormalities in Marfan Syndrome? (3)

  • leading cardiac cause of mortality and morbidity?
  • precautions to be instituted?
A
  1. Progressive aortic root dilatation (usually after 18 years of age)
  2. Mitral valve prolapse (leading cause of mortality and morbidity)
  3. Aortic valve insufficiency

Precautions:

  • no strenuous exercise since can lead to increased, irreversible aortic dilatation
  • may need to be started on beta blocker to reduce cardiac output and decrease aortic dilatation
  • ECHO at diagnosis and yearly afterwards
23
Q

What respiratory complications can be seen in Marfan syndrome? (3)

A
  1. Spontaneous pneumothorax due to long thoracic cage
    - cannot use blowing instruments, no scuba diving
  2. OSA due to facial structure
  3. Pectus excavatum (reduced pulmonary reserve)
24
Q

What gastrointestinal complication can be seen in Marfan Syndrome?

A

Increased risk for hernia

25
Q

What MSK complications can be seen in Marfan Syndrome? (6)

A
  1. Chest wall abnormalities: pectus excavatum or pectus carinatum
  2. Scoliosis: progressive and significant
  3. Thoracic kyphosis due to bone overgrowth or behavioural hunching
  4. Pes planus (flat feet)
  5. Protusio acetabulae: deep acetabulum of hips
  6. Dural ectaasia: stretching of dural sac in lumbrosacral region causing nerve entrapment, postural hypotension, low pressure headaches
26
Q

What are two clinical exam findings that are diagnostic of Marfan’s?

A
  1. Thumb sign: opposition of thumb across palm and close fist, thumb will stick out past palm
  2. Wrist sign: make fist around own wrist and will see pinkie overlap thumb
27
Q

What are the health supervision guidelines for patients with Marfan’s Syndrome?

A
  1. Cardiac: ECHO at diagnosis and yearly
  2. Ophtho: yearly assessment
  3. MSK: yearly exams, then from 6-18 yo, will be seen q6month for physical exam and bone age
  4. Genetic counseling
  5. Lifestyle and safety counseling
28
Q

What are red flags on history in a patient with bruising or injury? (6)

A
  1. No history of trauma or a vague history
  2. Any unexplained bruising in a baby < 6 months old
  3. A changing story
  4. A different history from different caregivers
  5. An injury not compatible with history of developmental stage
  6. Delay in seeking medical attention
29
Q

What is the most common cause of head injury in children < 1 years old?

A

Child abuse

30
Q

When should a skeletal survey be ordered in cases of suspected physical abuse?

A

< 2 years old to look for occult fractures

31
Q

Difference between TEN and SJS?

A

SJS < 10% skin involvement

TEN > 30% skin involvement

33
Q

When does colic typically start?

A

2 weeks and can last up to 4 months of age or longer

34
Q

Where are two places you must examine in any child with erythema multiforme rash?

A

Mucous membranes
Genital region

This is to rule out SJS and TEN

35
Q

What are acute complications of TEN?
What are long term complications of TEN?
Services to consult for patient with TEN?

A

Acute: sepsis, DIC, dehydration (fluid loss through skin), respiratory complications, pain

Long term: renal failure, liver failure, scarring, blindness, strictures (especially of GU)

Consultants: Derm, ophtho, urology (for urethral and vaginal strictures), ID

36
Q

What is the treatment for TEN?

A

Cyclophosphamide

IVIG

37
Q

What are the absolute contraindications to organ donation?

A
  1. HIV
  2. Hepatitis B & C
  3. HTLV infection
  4. Active systemic bacterial/fungal/viral infection
  5. Active malignancies
  6. Death with neurological disease of unestablished etiology
  7. Parkinson’s
  8. Subacute sclerosing panencephalopathy
  9. Progressive multifocal leukoencephalopathy
  10. Prion disease
  11. Rabies

Relative:

  1. Coroner’s case
  2. Hypotension/cardiac arrest
  3. Transmissable infections
  4. High risk behaviours
38
Q

What are the 3 hallmarks of informed choice?

A
  1. Appropriate information
  2. Decision-making capacity
  3. Voluntariness
39
Q

What are 6 recommendations to prevent dental caries in children?

A
  1. See dentist at 1 yo
  2. Brush teeth with first tooth (~6 months)
  3. Don’t put child to sleep with bottle
  4. Decrease juice consumption
  5. Fluoride tooth paste
  6. Fluoride varnish twice yearly
40
Q

Parents of child with unilateral cleft palate ask you what is the recurrence risk for their next pregnancy?

A

4%

41
Q

What is the age of consent for sexual activity in Canada?

A

Can consent if: 16 yo and there is NO relationship of trust, authority or dependency and doesn’t involve prostitution or pornography (for these, must be 18 yo)

  • exceptions:
  • 12-13 yo can consent with peers when age difference is NOT greater than 2 years
  • 14 and 15 yo can consent with peers when age difference is NOT greater than 5 years
  • < 12 yo cannot consent
42
Q

Clinical criteria for colic?

A
  1. Crying for > 3 hrs per day

2. At least 3 days per week for at least 1 week