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7th > Chapter 142- Kawasaki disease > Flashcards

Chapter 142- Kawasaki disease Flashcards

1
Q

Affects all blood vessels but primarily damages ____ sized muscular arteries

A

Medium

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2
Q

The ESR normalizes, usually at _____ weeks after the onset of fever

A

6 to 8

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3
Q

Least common observed clinical feature, only occurs in 75%

A

Cervical adenopathy

Unilateral often nonfluctuant

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4
Q

The most common cause of acquired heart disease in children in developed nations

A

Kawasaki disease

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5
Q

Highest incidence in ___ children

A

Asian

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6
Q

1 in 80 Japanese children develops Kawasaki by ____ years old

A

5

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7
Q

Treatment with IVIg reduces prevalence of coronary artery abnormalities from 25% in those treated with aspirin to ___%

A

5%

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8
Q

IVIg should be given during the first ___ days of fever

A

10

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9
Q

Illness of young children, specifically age range of

A

6 months to 5 years

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10
Q

Risk of KD is ___ fold higher in siblings; ___fold higher in Japanese

A

10; 10

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11
Q

Incidence of KD in children born to parents who had KD is ___ as high as in the general population

A

2x

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12
Q

3 forms of exanthem in Kawasaki disease

A

Morbiliform
Scarlatiniform
Targetoid

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13
Q

Classic periungual desquamation of fingers and toes does not begin until ____ after fever begins

A

2nd to 3rd week

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14
Q

Beau lines occur in ____ week after illness

A

3rd to 6th week

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15
Q

Criteria of Kawasaki syndrome:

Prolonged fever for 5 days or more +

A
  1. Bilateral, nonexudative Conjunctival injection
  2. Oral mucosal changes
  3. Changes of the hands, feet
  4. Rash
  5. Cervical adenopathy>/= 1.5 cm in diameter, unilateral
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16
Q

3 stages of kawasaki disease

A
  1. Acute febrile phase
  2. Subacute phase
  3. Convalescent phase
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17
Q

Oral ulcers are not a feature of Kawasaki disease.

True or False

A

True

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18
Q

More than 50% manifest with ____ which presents as tachycardia disproportionate to fever

A

Myocarditis

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19
Q

The ff are the most common arteries observed with aneurysms

A

Iliac, femoral, axillary

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20
Q

A functional polymorphism in ___ gene is associated with Kd susceptibility and risk of developing coronary artery abnormalities

A

ITPKC

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21
Q

3 linked patho physiologic processes characteristic of KD vasculopathy

A
  1. Neutrophilic necrotizing arteritis
  2. Subacute/ chronic necrotizing vasculitis
  3. Luminal myofibroblastic proliferation
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22
Q

Primary components of inflammatory infiltrate in acute Kd suggesting an immune response to intracellular pathogen with repiratory portal of entry

A

IgA plasma cells

CD8 T cells

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23
Q

A low white blood cell count with lymphocyte predominance would be usual for CBC of Kawasaki patients.

True or False

A

False, unusual

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24
Q

Thrombocytopenia is associated with more severe outcome

True or False

A

True

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25
Q

Characteristic of the subacute phase of Kawasaki

A

Thrombocytosis (> 1,000,000/mm3)

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26
Q

Thrombocytosis peaks in _____ after onset of fever

A

2nd to 3rd week

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27
Q

Patients with ___ and ___ are at higher risk for coronary artery disease

A

Anemia

Low albumin levels

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28
Q

A mild elevation of liver transaminases is commonly observed in acute Kawasaki.
True or False

A

True

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29
Q

Laboratory parameter used to follow clinical response in patients to monitor response to IVIg

A

CRP

IVIg causes transient elevation of ESR

30
Q

Labs like CBC, CRP/ESR should be performed at ___; repeated at ____ and ____ after onset to monitor for resolution of inflammation

A

Baseline

2 to 3 weeks; 6 to 8 weeks

31
Q

Peak time to detect coronary artery dilation

A

Subacute phase of illness (2-3 weeks after fever)

32
Q

Echocardiogram should be performed in all children with Kawasaki at

A

Baseline, 2-3 weeks after fever onset, 6-8 weeks after fever onset, 1 year after onset

33
Q

ECG often shows

A

Prolonged PR interval

Nonspecific ST and T wave changes

34
Q

Incomplete or Atypical KD criteria

Fever for 5 days or more with 2-3 clinical criteria +

A
  1. ESR >/= 40mm/h and/or CRP>/= 3.0 mg/dl
    —> 3 Compatible lab features, perform Echocardiogram
    —> less than 3 compatible lab features but with dilated coronary arteries >/=2.5
    —> less than 3 compatible lab features and less than 2.5 dilated artery score but with 3 supportive echocardiographic feature
  2. ESR < 40 mm/h and /or CRP < 3.0 mg/dl
35
Q

Compatible laboratory features

  1. Albumin _____
  2. Anemia for age
  3. Elevated ALT
  4. Platelet count ____ after the 7th day of illness
  5. WBC >/= ____
  6. Urinalysis with >/= ____/ HPO
A
  1. = 3.0 mg/dl
  2. > /= 450,000/mm3
  3. > /= 15,000/mm3
  4. > /= 10
36
Q

Supportive echocardiographic features:

A
  1. Lack of tapering
  2. Decreased LV function
  3. Mitral regurgitation
  4. Perocardial effusion
  5. Z scores of LAD or RCA of 2.0 to 2.5
37
Q

Best single test to differentiate Kawasaki and measles

A

Measles IgM antibody

38
Q

Treatment for Kawasaki

A

Single infusion of 2g/kg IVIg with aspirin 80-100mg/kg/day given every 6 hours

39
Q

High dose Aspirin should be continued until ___or until the patient has been afebrile for at least ___

A

14th day of illness; at least 2 days

40
Q

Aspirin at 80-100mg/kg is given for ___

And at 3-5 mg/kg/ day is given for ____

A

Antiinflammatory

Antithrombotic

41
Q

___ study showed that primary therapy with IVIg and tapering course if prednisolone over 2 to 3 weeks improved outcomes in high risk patients

A

RAISE

42
Q

Approximately ___% of acute KD patients are nonresponders

A

15

43
Q

Treatment for nonresponders

A
  1. Second dose of 2g/kg IVIg infusion
  2. High dose IV methylprednisolone OF for 3 days
  3. Infliximab
44
Q

Low dose aspirin should be discontinued at ___ after onset if all Echocardiograms, acute phase reactants are normal

A

6 to 8 weeks

45
Q

Edema of the face is more suggestive of Kawasaki than drug hypersensitivity reaction.
True or False

A

False

46
Q

If patient has epidemiologic risk factor, the ff diseases should be ruled out

A

Leptospirosis

Rocky Mountain fever

47
Q

Diagnosis is made

A

Clinically

48
Q

Characteristics of fever in Kawasaki disease

A

High spiking, intermittent, daily, lasts for 1-2 weeks

49
Q

Aneurysm rupture is less common, often occurs within first month of onset
True or False

A

True

50
Q

____ occur from thrombosis of an aneurysm, few months after onset

A

Myocardial infarction

51
Q

___ Japanese children develop Kawasaki disease

A

1 in 80

52
Q

Long term complications are confined to heart and vascular tree, primarily thrombosis and stenosis of major coronary arteries with __

A

Myocardial ischemia

53
Q

__% of untreated children develop coronary abnormalities leading to Mi and sudden death

A

25

54
Q

Damage in __ &; __ lead to loss of structural integrity of blood vessels with resultant aneurysm formation

A

Collagen

Elastic fibers

55
Q

__ are more commonly affected with ratio of 3:2

A

Boys

56
Q

Peak age of illness

A

9 to 11 mos of age

57
Q

There is __% recurrence rate

A

3

58
Q

Blood vessels in skin are not affected hence skin biopsy is not useful for diagnosis.
True or False

A

True

59
Q

Important clue to diagnosis of Kawasaki

A

Refuse to pick up objects or to walk

Extreme irritability

60
Q

Conjunctival injection in Kd is __, &; __ with __ sparing

A

Bilatera, nonexudative, limbal

61
Q

___ occurs during acute phase involves small joints but in subacute phase may involve knees, ankles

A

Arthritis

62
Q

Pericardial effusion occurs in subacute phase and resolves spontaneously.
True or False

A

False, acute phase

63
Q

Negative regulator of T cell activation

A

ITPKC gene

64
Q

__ are highly suggestive of viral etiology

A

Intracytoplasmic inclusion bodies

65
Q

Cbc result shows

A

Elevated wbc with neutrophil predominance

66
Q

Gallbladder hydrops with RUQ pain requires surgery

True or False

A

False, does not require

67
Q

Useful in evaluating the coronary arteries of teenagers and children with particularly severe disease

A

CT angiography or MR angiography

68
Q

Sterile pyuria is more typical of enterovirus infection

True or False

A

False, kawasaki

69
Q

Classical finding of SSSS

A

Painful skin

70
Q

Renal involvement with elevated creatinine levels are more likely to be observed in

A

TSS

71
Q

Fever recurs ff reduction of high dose aspirin

A

Juvenile rheumatoid arthritis

7th (23 decks)

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