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17 Cards in this Set
- Front
- Back
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Kawasaki Disease (KD) is an idiopathic ...
Predilection for the coronary arteries of ... and ... Discovered in Japan in 1967 by Tomisaku Kawasaki Sudden death from ... may occur many years later |
vasculitis
infants & children MI |
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Epidemiology:
-Incidence highest among Asians & Pacific Islanders -Boys outnumbered girls by 3:2 -76% of children were younger than ... years of age -In-hospital mortality was 0.17% -Virtually all deaths result from ... sequelae -Peak mortality occurs 15-45 days after the onset of ... -Ischemic heart disease in young adults may be “missed” KD |
5
cardiac fever |
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Causes and pathogenesis:
-Clinical and epidem. features suggest an ... source -Efforts unsuccessful to identify infectious agent -Immune response is oligoclonal or ...-driven -... plasma cells play a central role -Prominence of ... plasma cells in the respiratory tracts of fatal cases suggests a respiratory pathogen -Generalized ... affecting all blood vessels (coronary aa. most significantly effected) -Early influx of neutrophils followed by large mononuclear cells, lymphocytes and IgA plasma cells -Inflammation replaced by prog. fibrosis with scar formation |
infectious
antigen IgA IgA vasculitis |
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Criteria for Diagnosis of Kawasaki Disease:
Diagnosis is made if fever of ≥ ... days has occurred and ... of the following 5 criteria are noted: 1. Bilateral nonexudative ... injection 2. Changes in the ..., tongue, or oral mucosa (injection, drying, fissuring, red strawberry tongue) 3. Changes in the peripheral ... (edema, erythema, desquamation) 4. Polymorphous ... 5. Cervical ... (at least one node ≥ 1.5 cm in diameter) can be diagnosed w/ fewer than 4 of the principle criteria if coronary artery ... are detected by echocardiography. |
5
4 conjunctival lips extremities rash adenopathy aneurysms |
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Diagnosis:
... is the least common -Usually unilateral anterior cervical -Defined as ≥ 1.5cm in diameter -Often firm and non-tender with no erythema .../... in the first week KD pt’s often more ... than other febrile illnesses ... complaints occur in ~ 33% Hepatomegaly and jaundice can develop Up to 15% have abnormal findings on ... |
Cervical lymphadenopathy
Poly arthritis/arthralgia irritable GI cxray |
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Laboratory Evaluation:
-50% with > 15,000 ... -... with prolonged inflammation -Elevated ESR and CRP *ESR not useful after ... therapy -... - >500,000 (characteristic in subacute state) -40% with elevated LFT’s (liver function tests) -10% with ... bilirubin -... albumin associated with more severe disease -33% with sterile ... (looks like UTI but culture is negative) |
Leukocytosis
Anemia IVIG Thrombocytosis increased Decreased pyuria |
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Laboratory evaluation:
-50% have evidence of aseptic ... -Lab tests can provide diagnostic support for incomplete KD -... ≥ 3.0 mg/dl or ... >40 mm/hr is nearly universal in KD but unlikely in typical viral infections -KD unlikely if platelets and acute-phase reactants ... after 7 days of fever |
meningitis
CRP ESR normal |
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Incomplete or Atypical KD:
-Cases which do not fulfill clinical criteria and are diagnosed later by echo -More common in ... -... rarely form before day 10 -Consider in all pt’s with unexplained ... >5days and 2-3 principal diagnostic criteria -Lab criteria can support the diagnosis (Hb, CRP, ESR, albumin) -Consider doing an ... in any infant with fever lasting 7 days or more |
young infants
Aneurysms fever Echo |
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... of KD:
-Viral infections (eg, measles, adenovirus, enterovirus, Epstein-Barr virus) -Scarlet fever -Staphylococcal scalded skin syndrome -Toxic shock syndrome -Bacterial cervical lymphadenitis -Drug hypersensitivity -Stevens-Johnson syndrome -Juvenile idiopathic arthritis -Leptospirosis -Mercury hypersensitivity reaction (acrodynia) |
differential diagnosis
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Differential diagnosis:
-KD conjunctivitis is bulbar with no ... -Lip swelling and ... can be severe -Oral ulcers and exudative pharyngitis ... features of KD -Tense swelling of hands and feet distinctive often with later ... -... accentuation of the rash common with KD -Vesicles, bullae, petechiae, & purpura not seen in KD |
exudate
redness not desquamation Perineal |
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Cardiac imaging:
-... gold standard -Initial ... as soon as suspect diagnosis -Do not delay treatment -Proximal ... and ... most common sites for aneurysms |
Echo
Echo RCA and LAD |
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KD Treatment:
-High dose ... and ... -Faster fever resolution and reduced coronary abnormalities by 85% -Appear to have additive anti-inflammatory effect -Acute phase ... 80-100mg/kg divided QID x 14 days -... 3-5mg/kg/day until negative ... at 6-8 weeks -Pt’s with coronary abn may require ... indefinitely -ASA alone ... lower the risk of coronary abnormalities |
ASA and IVIG
ASA ASA Echo ASA does not |
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... – worry about this w/ kids taking aspirin
-Prodromal URI , influenza A or B illness or Varicella -History of salicylate ingestion -Vomiting -Lethargy, drowsiness progressing to semi-coma -Elevated AST, hyperammonemia, nml or slightly increased bilirubin, prolonged prothrombin time -Microvesicular steatosis of the liver, kidneys, brain -Absence of other metabolic diseases -All long-term ASA treated KD patients need routine flu shots |
Reye Syndrome
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KD Treatment:
-... 2g/kg single infusion -Start within 10 days of first day of fever when possible -Treat after 10 days if persistent fever or inflammation -Avoid measles & varicella immunization for 11mos after ... -5% develop coronary dilation -1% develop giant aneurysms, >8mm -Steroids may decrease the rate of ... retreatment -10% fail to respond to initial IVIG -? monoclonal Ab (Infliximab); ? Cytotoxic agents |
IVIG
IVIG IVIG |
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Long-term outcomes:
-... occur in 20-25% of untreated children *50% of them resolve after 1-2 years -Smaller ... have greater chance of regression -... from thrombotic occlusion is principal cause of death *MI usually occurs in the first year -Development of new ... unlikely after 1-2 months -... at diagnosis, 2 & 6 weeks after onset -Evidence suggests long-term endothelial dysfunction -Treatment can be individualized according to risk level |
CAA (coronary artery aneurysm)
aneurysms MI aneurysms Echo |
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look at slide 27 and 28
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ok
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-Remember risk of ... with ASA and viral illness
-Remember ... in children with prolonged fever and young adults presenting with chest pain. -... can be differentiated by: Increased irritability; Perineal accentuated rash; Hand and feet involvement; and dramatically elevated inflam. markers -Characteristics suggesting ... include: exudative conjunctivitis, exudative pharyngitis, discrete intraoral lesions, bullous or vesicular rash, or generalized adenopathy. -Awaiting identification of an etiologic agent |
Reye Syndrome
Kawasaki’s disease KD disease other than KD |
