Kawasaki disease


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mucocutaneous

 [mu″ko-ku-ta´ne-us]
pertaining to mucous membrane and skin.
mucocutaneous lymph node syndrome Kawasaki's disease.

mu·co·cu·ta·ne·ous lymph node syn·drome

a systemic vasculitis of unknown origin that occurs primarily in children under 8 years of age. Symptoms include a fever lasting more than 5 days; polymorphic rash; erythematous, dry, cracking lips; conjunctival injection; swelling of the hands and feet; irritability; adenopathy; and a perineal desquamative rash. Approximately 20% of untreated patients may develop coronary artery aneurysms. As the child recovers from the illness, thrombocytosis and peeling of the fingertips occur.

Kawasaki disease

Kawasaki syndrome

An acute idioapathic febrile illness that primarily affects children under age 5, especially males, and has a winter-spring seasonality.

Clinical findings
Fever; cervical lymphadenopathy; palmoplantar and mucosal erythaema and oedema; conjunctivitis; gingivitis; maculoerythematous glove-and-sock rash over the hands and feet, which becomes hard, oedematous and sloughs; aneurysms of small- and medium-sized coronary, occasionally peripheral, arteries, with arteritis. Other findings include conjunctival oedema, upper respiratory tract oedema, pharyngeal injection and truncal lymphadenopathy.
 
Aetiology
Uncertain; various bacteria have been implicated, though none definitively.
 
Lab
Increased ESR, CRP, complement, immunoglobulins.
 
Management
Gammaglobulin in IV bolus, aspirin IV.
 
Prognosis
May cause sudden death; 1–5% die of disease.

Kawasaki syndrome case definition (CDC)
Fever of 5 days’ duration (or fever post-IV immunoglobulin if given before 5th day of fever), and 4 of following 5 signs:
• Rash;     
• Cervical lymphadenopathy (1.5+ cm in diameter);
• Bilateral conjunctival injection;   
• Oral mucosal changes—erythaema, fissuring and xerostomia, “strawberry tongue”;    
• Peripheral extremity changes—e.g., acral erythema or oedema, periungual and/or generalised desquamation, polymorphous exanthematous rash.

Kawasaki disease

Mucocutaneous lymph node syndromeA disease of children < age 5 that often follows a 1-2 wk prodrome Etiology Uncertain; bugs implicated include Proprionibacterium acnes or retroviruses, none definitively Clinical Fever, cervical lymphadenopathy, palmoplantar and mucosal erythema and edema, conjunctivitis, gingivitis, maculoerythematous glove-and-sock rash over the hands and feet which becomes hard, swollen–edematous, and sloughs, aneurysms of small and medium-sized coronary, occasionally peripheral arteries, with arteritis Lab ↑ ESR, CRP, complement, Igs Management Igs in IV bolus, aspirin IV Prognosis Possibly sudden death;
1-5% die of disease
Kawasaki disease*  
1. Fever of > 5 days
.
2. Bilateral ocular conjunctival injection
.
3. One or more changes of oral mucosa, including erythema, fissuring and xerostomia, conjunctival edema, mucosal edema of upper respiratory tract, eg pharyngeal injection, dry, fissured lips and 'strawberry tongue'
.
4. One or more changes of extremities, including acral erythema or edema, periungual and/or generalized desquamation, polymorphous exanthematous rash, truncal and cervical lymphadenopathy  
*CDC definition, requires 4+ of the above
.

Ka·wa·sa·ki dis·ease

, Kawasaki syndrome (kă-wă-sah'kē di-zēz', sin'drōm)
A systemic vasculitis of unknown origin that occurs primarily in children younger than 8 years of age. Symptoms include a fever lasting more than 5 days; polymorphic rash; erythematous, dry, cracking lips; conjunctival injection; swelling of the hands and feet; irritability; adenopathy; and a perineal desquamative rash. Approximately 20% of untreated patients may develop coronary artery aneurysms. Treatment includes oral aspirin at high dosage, immune globulin administered intravenously, and supportive care. As the child recovers from the illness, thrombocytosis and peeling of the fingertips occur.
Synonym(s): mucocutaneous lymph node syndrome.

Kawasaki disease

A world-wide disease of infants and young children that causes fever, swollen lymph nodes, a measles-like rash, red eyes, inflamed tongue, dry cracking lips, peeling skin and, in just under half the cases, local widening (ANEURYSMS) in the CORONARY arteries. These are usually transient but 10% have long-term involvement of the coronaries. No causal agent has been discovered but high doses of immune globulin reduce the risk of coronary artery damage. Aspirin is also effective. Fewer than 1% of affected children die from the disease and most make a complete recovery. The condition was first recognized in 1967 by the Japanese paediatrician Dr Tomisaku, in the port of Kawasaki.

Kawasaki,

Tomisaku, 20th century Japanese pediatrician.
Kawasaki disease - a polymorphous erythematous febrile, sometimes epidemic, disease of unknown etiology occurring in children. Synonym(s): Kawasaki syndrome; mucocutaneous lymph node syndrome
Kawasaki syndrome - Synonym(s): Kawasaki disease
References in periodicals archive ?
2000) Result of surgical treatments in patients with coronary-arterial obstructive disease after Kawasaki disease.
Update on etio and immunopathogenesis of Kawasaki disease.
The study contributes to the growing body of research highlighting the link between Kawasaki disease and the environment, says Xavier Rodo, an atmospheric scientist who studies Kawasaki disease at the Catalan Institute of Climate Sciences in Barcelona, Spain.
A Severe Form of Kawasaki Disease Presenting with Only Fever and Cervical Lymphadenopathy at Admission.
Diagnosis, treatment and long-term management of Kawasaki disease: a statement for health professionals from the Committee on rheumatic fever, endocarditis, and Kawasaki disease, Council on cardiovascular disease in the young, American Heart Association.
The indicators of resistance to IVIG in Kawasaki disease include male gender, being younger than one year old, administration of IVIG after the 10th day, thrombocytopenia, low albumin level, low hemoglobin level, low sodium level, persistent increased CRP after IVIG infusion and persistent low serum albumin level after IVIG infusion.
Kawasaki disease should be considered in any child with fever for five or more days with no known cause.
Diagnosis, treatment and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association.
A diagnosis of incomplete Kawasaki disease was made in a 10-month-old female infant on the basis of fever, widespread erythematous rash of both lower extremities, swelling in both hands and feet (Fig.
Two sets of monozygotic (MZ) twins with Kawasaki disease (KD) from two different families are reported.
Kawasaki disease, a serious childhood illness that causes inflammation of blood vessels throughout the body, has been linked with early-onset and accelerated atherosclerosis, a leading cause of heart disease in adults.
Morbidity prevalence rate of Kawasaki disease assessed by single cross-sectional history-taking.

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