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7th > Chapter 142- Kawasaki disease > Flashcards

Chapter 142- Kawasaki disease Flashcards

(71 cards)

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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
Characteristic of the subacute phase of Kawasaki
Thrombocytosis (> 1,000,000/mm3)
26
Thrombocytosis peaks in _____ after onset of fever
2nd to 3rd week
27
Patients with ___ and ___ are at higher risk for coronary artery disease
Anemia | Low albumin levels
28
A mild elevation of liver transaminases is commonly observed in acute Kawasaki. True or False
True
29
Laboratory parameter used to follow clinical response in patients to monitor response to IVIg
CRP | IVIg causes transient elevation of ESR
30
Labs like CBC, CRP/ESR should be performed at ___; repeated at ____ and ____ after onset to monitor for resolution of inflammation
Baseline | 2 to 3 weeks; 6 to 8 weeks
31
Peak time to detect coronary artery dilation
Subacute phase of illness (2-3 weeks after fever)
32
Echocardiogram should be performed in all children with Kawasaki at
Baseline, 2-3 weeks after fever onset, 6-8 weeks after fever onset, 1 year after onset
33
ECG often shows
Prolonged PR interval | Nonspecific ST and T wave changes
34
Incomplete or Atypical KD criteria | Fever for 5 days or more with 2-3 clinical criteria +
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
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
1. = 3.0 mg/dl 4. >/= 450,000/mm3 5. >/= 15,000/mm3 6. >/= 10
36
Supportive echocardiographic features:
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
Best single test to differentiate Kawasaki and measles
Measles IgM antibody
38
Treatment for Kawasaki
Single infusion of 2g/kg IVIg with aspirin 80-100mg/kg/day given every 6 hours
39
High dose Aspirin should be continued until ___or until the patient has been afebrile for at least ___
14th day of illness; at least 2 days
40
Aspirin at 80-100mg/kg is given for ___ | And at 3-5 mg/kg/ day is given for ____
Antiinflammatory | Antithrombotic
41
___ study showed that primary therapy with IVIg and tapering course if prednisolone over 2 to 3 weeks improved outcomes in high risk patients
RAISE
42
Approximately ___% of acute KD patients are nonresponders
15
43
Treatment for nonresponders
1. Second dose of 2g/kg IVIg infusion 2. High dose IV methylprednisolone OF for 3 days 3. Infliximab
44
Low dose aspirin should be discontinued at ___ after onset if all Echocardiograms, acute phase reactants are normal
6 to 8 weeks
45
Edema of the face is more suggestive of Kawasaki than drug hypersensitivity reaction. True or False
False
46
If patient has epidemiologic risk factor, the ff diseases should be ruled out
Leptospirosis | Rocky Mountain fever
47
Diagnosis is made
Clinically
48
Characteristics of fever in Kawasaki disease
High spiking, intermittent, daily, lasts for 1-2 weeks
49
Aneurysm rupture is less common, often occurs within first month of onset True or False
True
50
____ occur from thrombosis of an aneurysm, few months after onset
Myocardial infarction
51
___ Japanese children develop Kawasaki disease
1 in 80
52
Long term complications are confined to heart and vascular tree, primarily thrombosis and stenosis of major coronary arteries with __
Myocardial ischemia
53
__% of untreated children develop coronary abnormalities leading to Mi and sudden death
25
54
Damage in __ &; __ lead to loss of structural integrity of blood vessels with resultant aneurysm formation
Collagen | Elastic fibers
55
__ are more commonly affected with ratio of 3:2
Boys
56
Peak age of illness
9 to 11 mos of age
57
There is __% recurrence rate
3
58
Blood vessels in skin are not affected hence skin biopsy is not useful for diagnosis. True or False
True
59
Important clue to diagnosis of Kawasaki
Refuse to pick up objects or to walk | Extreme irritability
60
Conjunctival injection in Kd is __, &; __ with __ sparing
Bilatera, nonexudative, limbal
61
___ occurs during acute phase involves small joints but in subacute phase may involve knees, ankles
Arthritis
62
Pericardial effusion occurs in subacute phase and resolves spontaneously. True or False
False, acute phase
63
Negative regulator of T cell activation
ITPKC gene
64
__ are highly suggestive of viral etiology
Intracytoplasmic inclusion bodies
65
Cbc result shows
Elevated wbc with neutrophil predominance
66
Gallbladder hydrops with RUQ pain requires surgery | True or False
False, does not require
67
Useful in evaluating the coronary arteries of teenagers and children with particularly severe disease
CT angiography or MR angiography
68
Sterile pyuria is more typical of enterovirus infection | True or False
False, kawasaki
69
Classical finding of SSSS
Painful skin
70
Renal involvement with elevated creatinine levels are more likely to be observed in
TSS
71
Fever recurs ff reduction of high dose aspirin
Juvenile rheumatoid arthritis

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