Chapter 142- Kawasaki disease Flashcards
Affects all blood vessels but primarily damages ____ sized muscular arteries
Medium
The ESR normalizes, usually at _____ weeks after the onset of fever
6 to 8
Least common observed clinical feature, only occurs in 75%
Cervical adenopathy
Unilateral often nonfluctuant
The most common cause of acquired heart disease in children in developed nations
Kawasaki disease
Highest incidence in ___ children
Asian
1 in 80 Japanese children develops Kawasaki by ____ years old
5
Treatment with IVIg reduces prevalence of coronary artery abnormalities from 25% in those treated with aspirin to ___%
5%
IVIg should be given during the first ___ days of fever
10
Illness of young children, specifically age range of
6 months to 5 years
Risk of KD is ___ fold higher in siblings; ___fold higher in Japanese
10; 10
Incidence of KD in children born to parents who had KD is ___ as high as in the general population
2x
3 forms of exanthem in Kawasaki disease
Morbiliform
Scarlatiniform
Targetoid
Classic periungual desquamation of fingers and toes does not begin until ____ after fever begins
2nd to 3rd week
Beau lines occur in ____ week after illness
3rd to 6th week
Criteria of Kawasaki syndrome:
Prolonged fever for 5 days or more +
- Bilateral, nonexudative Conjunctival injection
- Oral mucosal changes
- Changes of the hands, feet
- Rash
- Cervical adenopathy>/= 1.5 cm in diameter, unilateral
3 stages of kawasaki disease
- Acute febrile phase
- Subacute phase
- Convalescent phase
Oral ulcers are not a feature of Kawasaki disease.
True or False
True
More than 50% manifest with ____ which presents as tachycardia disproportionate to fever
Myocarditis
The ff are the most common arteries observed with aneurysms
Iliac, femoral, axillary
A functional polymorphism in ___ gene is associated with Kd susceptibility and risk of developing coronary artery abnormalities
ITPKC
3 linked patho physiologic processes characteristic of KD vasculopathy
- Neutrophilic necrotizing arteritis
- Subacute/ chronic necrotizing vasculitis
- Luminal myofibroblastic proliferation
Primary components of inflammatory infiltrate in acute Kd suggesting an immune response to intracellular pathogen with repiratory portal of entry
IgA plasma cells
CD8 T cells
A low white blood cell count with lymphocyte predominance would be usual for CBC of Kawasaki patients.
True or False
False, unusual
Thrombocytopenia is associated with more severe outcome
True or False
True
Characteristic of the subacute phase of Kawasaki
Thrombocytosis (> 1,000,000/mm3)
Thrombocytosis peaks in _____ after onset of fever
2nd to 3rd week
Patients with ___ and ___ are at higher risk for coronary artery disease
Anemia
Low albumin levels
A mild elevation of liver transaminases is commonly observed in acute Kawasaki.
True or False
True
Laboratory parameter used to follow clinical response in patients to monitor response to IVIg
CRP
IVIg causes transient elevation of ESR
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
Peak time to detect coronary artery dilation
Subacute phase of illness (2-3 weeks after fever)
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
ECG often shows
Prolonged PR interval
Nonspecific ST and T wave changes
Incomplete or Atypical KD criteria
Fever for 5 days or more with 2-3 clinical criteria +
- 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 - ESR < 40 mm/h and /or CRP < 3.0 mg/dl
Compatible laboratory features
- Albumin _____
- Anemia for age
- Elevated ALT
- Platelet count ____ after the 7th day of illness
- WBC >/= ____
- Urinalysis with >/= ____/ HPO
- = 3.0 mg/dl
- > /= 450,000/mm3
- > /= 15,000/mm3
- > /= 10
Supportive echocardiographic features:
- Lack of tapering
- Decreased LV function
- Mitral regurgitation
- Perocardial effusion
- Z scores of LAD or RCA of 2.0 to 2.5
Best single test to differentiate Kawasaki and measles
Measles IgM antibody
Treatment for Kawasaki
Single infusion of 2g/kg IVIg with aspirin 80-100mg/kg/day given every 6 hours
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
Aspirin at 80-100mg/kg is given for ___
And at 3-5 mg/kg/ day is given for ____
Antiinflammatory
Antithrombotic
___ study showed that primary therapy with IVIg and tapering course if prednisolone over 2 to 3 weeks improved outcomes in high risk patients
RAISE
Approximately ___% of acute KD patients are nonresponders
15
Treatment for nonresponders
- Second dose of 2g/kg IVIg infusion
- High dose IV methylprednisolone OF for 3 days
- Infliximab
Low dose aspirin should be discontinued at ___ after onset if all Echocardiograms, acute phase reactants are normal
6 to 8 weeks
Edema of the face is more suggestive of Kawasaki than drug hypersensitivity reaction.
True or False
False
If patient has epidemiologic risk factor, the ff diseases should be ruled out
Leptospirosis
Rocky Mountain fever
Diagnosis is made
Clinically
Characteristics of fever in Kawasaki disease
High spiking, intermittent, daily, lasts for 1-2 weeks
Aneurysm rupture is less common, often occurs within first month of onset
True or False
True
____ occur from thrombosis of an aneurysm, few months after onset
Myocardial infarction
___ Japanese children develop Kawasaki disease
1 in 80
Long term complications are confined to heart and vascular tree, primarily thrombosis and stenosis of major coronary arteries with __
Myocardial ischemia
__% of untreated children develop coronary abnormalities leading to Mi and sudden death
25
Damage in __ &; __ lead to loss of structural integrity of blood vessels with resultant aneurysm formation
Collagen
Elastic fibers
__ are more commonly affected with ratio of 3:2
Boys
Peak age of illness
9 to 11 mos of age
There is __% recurrence rate
3
Blood vessels in skin are not affected hence skin biopsy is not useful for diagnosis.
True or False
True
Important clue to diagnosis of Kawasaki
Refuse to pick up objects or to walk
Extreme irritability
Conjunctival injection in Kd is __, &; __ with __ sparing
Bilatera, nonexudative, limbal
___ occurs during acute phase involves small joints but in subacute phase may involve knees, ankles
Arthritis
Pericardial effusion occurs in subacute phase and resolves spontaneously.
True or False
False, acute phase
Negative regulator of T cell activation
ITPKC gene
__ are highly suggestive of viral etiology
Intracytoplasmic inclusion bodies
Cbc result shows
Elevated wbc with neutrophil predominance
Gallbladder hydrops with RUQ pain requires surgery
True or False
False, does not require
Useful in evaluating the coronary arteries of teenagers and children with particularly severe disease
CT angiography or MR angiography
Sterile pyuria is more typical of enterovirus infection
True or False
False, kawasaki
Classical finding of SSSS
Painful skin
Renal involvement with elevated creatinine levels are more likely to be observed in
TSS
Fever recurs ff reduction of high dose aspirin
Juvenile rheumatoid arthritis
