mucocutaneous lymph node syndrome


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Related to mucocutaneous lymph node syndrome: Kawasaki syndrome

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.

mucocutaneous lymph node syndrome (MLNS)

an acute febrile illness, primarily of young children, characterized by inflamed mucous membranes of the mouth; "strawberry tongue"; cervical lymphadenopathy; polymorphous rash on the trunk; and edema, erythema, and desquamation of the skin on the extremities. Other commonly associated findings include arthralgia, diarrhea, otitis, pneumonia, photophobia, meningitis, and electrocardiographic changes. The cause is unknown. No clear-cut environmental, seasonal, or geographic factors have been discovered, and person-to-person transmission is unproved. A genetic predisposition has been indicated. Treatment includes IV gammaglobulin; aspirin in large doses, which may be prescribed over a long period; and supportive care. Also called Kawasaki disease.
observations The acute stage of the disease begins with a remittent fever (103° F to 105° F) accompanied by extreme irritability, lethargy, and intermittent colicky abdominal pain. The fever typically lasts 7 days to 2 weeks and is unresponsive to antibiotics. After 1 to 2 days, bilateral conjunctivitis occurs. Within 5 days, there is erythema and edema of the hands and feet, a macular rash on the trunk and perineum, strawberry tongue, fissuring of the lips, and reddened pharynx. Cervical lymphadenopathy is present. Ten to 25 days after onset, fever resolves and symptoms diminish. Irritability, anorexia, and conjunctivitis remain. There is peeling of the hands and feet, arthritis, arthralgia, and thrombocytosis. The greatest risk for coronary artery aneurysm occurs during this time. During convalescence, all clinical signs have resolved, but erythrocyte sedimentation rate is still abnormal. ESR blood values return to normal about 6 to 8 weeks after onset. Clinical exam reveals a history of fever with four of five of the following conditions: conjunctivitis, changes in mucosa of oropharynx, edema and erythema of hands and feet, nonvesicular truncal rash, and lymphadenopathy. Blood studies reveal elevated WBCs and platelets; elevated ESR; positive C-reactive protein; and elevated ALT and AST. Chest x-rays may reveal pulmonary infiltrates. An echocardiogram may show depressed left ventricular function. Echocardiograms are also useful to monitor cardiac status during the course of the disease. Complications include coronary arteritis, coronary artery aneurysms, and thrombotic occlusion of an aneurysm leading to a myocardial infarction and possible death. However, prognosis with treatment is excellent, with a mortality rate of less than 0.1%.
interventions Intravenous immunoglobulin is the primary treatment to reduce inflammation in the acute stage. Aspirin therapy is used until temperatures return to normal. Corticosteroids increase possibility of aneurysm and are contraindicated in treatment of MLNS. Anticoagulant and antiplatelet therapy may be used if there is evidence of coronary thrombosis. If the child develops cardiac complications, percutaneous transluminal coronary angioplasty, coronary bypass grafting, or cardiac transplant may be required.
nursing considerations Nursing care is primarily supportive and includes careful monitoring of cardiac status, careful fluid replacement, I & O, comfort measures (lubrication for lips, lotion for skin, and cool cloths), quiet soothing environment, and parental support in attempting to console an irritable child. Parental education is needed about possible cardiac complications. Caregiver instruction in CPR should be provided if cardiac complications are present.
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mucocutaneous lymph node syndrome

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.

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.

Mucocutaneous lymph node syndrome (MLNS)

Mucocutaneous lymph node syndrome, another name for Kawasaki syndrome. The name comes from the key symptoms of the disease, which involve the mucous membranes of the mouth and throat, the skin, and the lymph nodes.
Mentioned in: Kawasaki Syndrome

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

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

(myū'kō-kyū-tā'nē-ŭs limf nōd sin'drōm)
Systemic vasculitis of unknown origin that occurs primarily in children younger than 8 years of age.
Synonym(s): Kawasaki disease.