Kawasaki disease

The Kawasaki syndrome or mucocutaneous lymph node syndrome ( MCLS ) is an acute, febrile, systemic disease that is characterized by blood vessel inflammation ( vasculitis necrotising ) of the small and medium-sized arteries. In addition, a systemic inflammation in many organs is present. The cause is unknown, is suspected infective origin, which is favored by a hereditary basis. The Kawasaki syndrome affects mostly infants and imitated in the initial appearance of infectious diseases such as measles or scarlet fever.

Epidemiology

In Germany the disease per year, about 9 out of 100,000 children under 5 years, while the incidence of a Kawasaki disease in Japan is in the same age group at about 185 of 100,000. 75 % of all affected children are younger than five years, very often fall ill patients between the first and second year of life. Boys are times as often as girls affected by the disease about one and a half. There is a seasonal peak in winter and spring.

Clinical manifestations

This is followed by successive three phases:

Main symptoms

In addition to symptoms

Methods of investigation

The diagnosis of a complete Kawasaki disease is made clinically and requires the fever and 4 of 5 of the other criteria and the exclusion of other diseases with similar symptoms. Then, the diagnosis can be made ​​within five days of fever. Children who do not meet these criteria are diagnosed as so-called incomplete Kawasaki disease, below mentioned accessory symptoms and / or laboratory findings apply partially or predominantly. This occurs more often in children under one year and is here because of the increased risk of Koronararterienaneurysmen particularly problematic.

Laboratory findings can not prove or disprove the disease; they can make more likely or less likely the diagnosis in ambiguous clinical signs:

New markers in urine. American researchers have now been able to find markers in the urine of affected children, which make the disease more quickly safely diagnosed. The markers are the filamin C ( is from necrotic cardiac and skeletal muscle cells excreted in the urine) and meprin A ( enzyme of the inflammatory response ). The diagnosis was made on the basis of these markers in a study of 107 patients with 98 % accuracy.

Differential diagnoses

Viral and bacterial diseases, Leptospira, systemic-onset juvenile idiopathic arthritis, polyarteritis nodosa, mercury poisoning. In incomplete Kawasaki disease, there are numerous other possibilities for confusion. In fever of unknown cause should be early thought of the differential diagnosis Kawasaki syndrome and in addition to the laboratory tests also an echocardiogram be performed with examination of the coronary arteries especially when small child.

Complications

• Inflammation of the coronary arteries ( coronary arteries ) with aneurysm formation
• Occurring or after years stenosis

Coronary vessels

The long-term course of the disease depends on whether changes in the coronary arteries arise. About 25 % of untreated children develop one or more aneurysms. In this case, the daily intake of a slight blood thinner such as aspirin (100 mg ) in children recommended. About half of these aneurysms are formed within one year back. In approximately 20 % of patients develop in the course of years to stenoses, which in turn lead untreated in almost half the cases of myocardial infarction.

Therapy

The disease is commonly treated in hospital. Therapy results in a reduction of inflammation, and the prevention of coronary artery aneurysms goal which. Mostly in the 2 -3 Week arise. It could be shown that the occurrence of coronary vascular lesions of 25% could be reduced to 2 to 4% by therapy. Therefore, initiation of treatment before the tenth day is crucial for a favorable outcome. The initial treatment is:

• Immunoglobulins: 2 g / kg body weight in 12 hours as an infusion, after a very early gift and unsatisfactory response possibly one more time.
• Acetylsalicylic acid (ASA ) until resolution of acute inflammation in high doses (30 to 100 mg / kg bw / day) for 14 days. The dose is controversial.
• Whether cortisone- like drugs in " treatment failures " are helpful, is still the subject of research, they do show at least an additive effect. A cortisone pulse treatment was no better than placebo.

Prolonged therapy following is recommended with aspirin 3 to 5 mg / (kg * day) for about six to eight weeks, further measures are based on the occurrence of coronary aneurysms: As long as an aneurysm exists, aspirin should be discontinued in the low dosage not be. Has a large aneurysm with constrictions formed blood clotting where appropriate, should be inhibited by other drugs such as warfarin. Furthermore, may come bypasses etc. in question.

Long-term expectations

The acute illness usually heals without complications. If complications occur in the coronary arteries, according to current estimates the risk of arteriosclerosis is increased due to altered vessel wall. For this reason, Kawasaki patients should be followed up long-term cardiology. Another symptom is a reduced efficiency of the body during exercise ( sports).

History

The Kawasaki syndrome has been reported in Japan since 1961 and described as a disease in 1967 by the physician Tomisaku Kawasaki. A study carried out in 2011 brings the disease associated with air flows from the sea.