Kawasaki syndrome is an acute febrile illness characterized primarily by inflammatory reactions of the arterial blood vessels with multiple organ involvement and occurs in infancy (up to 5 years of age). Kawasaki syndrome is most common in Japan and, with increasing frequency, in Germany (approximately 9 per 100,000 children).
What is Kawasaki syndrome?
Kawasaki syndrome (also known as mucocutaneous lymph node syndrome) is an acute febrile illness that occurs primarily in young children, particularly between the ages of 2 and 5 years, and is associated with vasculitis, an inflammation of the smaller and medium-sized blood vessels. In most cases, Kawasaki syndrome, which initially resembles other infectious diseases such as scarlet fever or measles, is additionally manifested by a systemic inflammation of the internal organs. In addition to the sudden onset of fever, swelling of the lymph nodes in the neck and throat (cervical lymphadenopathy), nonpurulent conjunctivitis (inflammation of the conjunctiva), stomatitis (inflammation of the oral mucosa) with strawberry tongue or varnish tongue, polymorphic polymorphism (polymorphonucleosis), and a severe inflammation of the internal organs. Lacquer tongue, a polymorphous exanthema (nonpruritic multiform rash on the trunk), and scaly erythema (redness) on the hands and feet characteristic of Kawasaki syndrome.
Causes
The underlying causes of Kawasaki syndrome have not yet been determined. It is suspected that Kawasaki syndrome is etiologically due to infection with as yet unidentified bacteria or viruses to whose superantigens (toxic-acting proteins of the pathogens) the immune defenses of the affected children react with pronounced inflammatory reactions as a result of a genetically determined defect. A genetic predisposition (disposition) is also supported by the significantly higher incidence (number of new cases) of Kawasaki syndrome in Japan compared with Europe or North America.
Symptoms, complaints, and signs
Infants and young children who have Kawasaki syndrome suffer from severe inflammation of blood vessels throughout the body. The inflammation causes symptoms such as fever and rash. Typical of the disease is a gradual increase in body temperature, usually associated with chills, faintness and a general feeling of malaise. The rash takes the form of painful redness, which can occur in any part of the body and occasionally forms ulcers or cysts. This may be accompanied by conjunctivitis and swelling of the mucous membranes in the mouth and throat. Red lips and a reddish tongue, also called strawberry tongue, are also characteristic. With early treatment, affected children usually recover relatively quickly. In severe cases, however, the rash can leave scars or cause permanent sensitivity problems. If no treatment is given, there is a danger to life. Then the skin lesions worsen and the fever increases. Eventually, complications of the heart may occur, resulting in the death of the child. Symptoms usually appear between the ages of one and eight. However, adolescents and young adults may also be affected. Kawasaki syndrome is particularly common in boys.
Diagnosis and course
Kawasaki syndrome can be diagnosed by its characteristic symptoms. In addition to the acute onset of high fever, at least four of the main symptoms of the disease must be detectable to assume Kawasaki syndrome. In addition, similar infectious diseases such as measles or scarlet fever should be excluded for differential diagnosis. The diagnosis is confirmed by a blood analysis. So-called autoantibodies (cANCA), antibodies directed against the body’s own antigens, can be detected serologically in Kawasaki syndrome. An increased leukocyte count (white blood cells) as well as an increased CRP value (C-reactive protein) also indicate a possible Kawasaki syndrome as inflammation markers. Echocardiography (cardiac ultrasound) should be performed to rule out involvement of the coronary vessels (arterial coronary vessels) or cardiac aneurysm (bulging of the arterial coronary vessels).If left untreated, Kawasaki syndrome can lead to aneurysm of the heart, which can result in life-threatening secondary diseases such as myocarditis (inflammation of the heart muscle), infarcts, pericarditis (inflammation of the pericardium), and cardiac arrhythmias, which have a significant impact on long-term lethality (mortality rate). With timely diagnosis and initiation of therapy, the prognosis is good and the disease usually heals without complications.
Complications
Due to Kawasaki syndrome in babies and children, most cases develop a very severe fever. Those affected also suffer from a red coloration of the skin, lips, and tongue. Likewise, the mucous membranes in the throat and mouth swell, causing discomfort in the intake of food and fluids. It is not uncommon for Kawasaki syndrome in babies and children to also be associated with a rash over the entire body and inflammation of the conjunctiva. The patient’s quality of life is significantly limited and reduced by this syndrome. Without treatment, the syndrome can also cause discomfort and inflammation of the heart, which can eventually lead to the death of the affected person. During the treatment itself, there are usually no particular complications and the course of the disease is always positive. This is done with the help of medications that considerably limit the complaints and symptoms. Successful treatment does not lead to further complications or a reduced life expectancy. Consequential damage also does not occur with successful treatment of Kawasaki syndrome in babies and children.
When should you see a doctor?
A visit to the doctor is necessary in case of a persistent or rising fever. Redness of the skin, an increase in heart activity, and changes in the appearance of the skin, should be assessed and examined by a doctor. Children up to 5 years of age suffering from sudden acute symptoms leading to severe health impairment should be treated by a physician as soon as possible. Since Kawasaki syndrome in babies and children can lead to premature death in severe cases without medical attention, a visit to the doctor is recommended at the first irregularities. Red discoloration of the lips and tongue, resembling a strawberry color, must be investigated. Swelling of the lymph, pain of the lymph nodes, or a change in the mucous membranes of the mouth and throat, should be presented to a physician. If conjunctivitis occurs, a visit to the doctor must be made. In case of disturbances of the heart rhythm, a doctor should be consulted immediately. Damage to the heart muscle may occur, with serious consequences. If the child refuses to eat and drink enough fluids, the organism may be undernourished. A visit to the doctor is required immediately to prevent a life-threatening condition from developing. If the child shows behavioral abnormalities or an increased need for sleep, a doctor should be consulted.
Treatment and therapy
Therapeutic measures in Kawasaki syndrome are aimed primarily at reducing inflammatory reactions and preventing aneurysms in the coronary arteries, for which there is an increased risk of manifestation in the second and third weeks. For this purpose, immunoglobulins, which play an important role in the body’s defense and cannot be synthesized independently to a sufficient extent by children affected by Kawasaki syndrome, are infused (2 g/kg body weight over 12 hours). The therapeutic measure may have to be repeated if there is an insufficient response on the part of the affected child. In addition, high-dose acetylsalicylic acid (30 to 100 mg/kg body weight per day) is used to reduce acute inflammation and to reduce fever. As long as the risk of coronary aneurysm exists or is present, acetylsalicylic acid should continue to be taken in lower doses (2 to 4 mg/kg body weight per day) because the substance has an anticoagulant effect. In the presence of a pronounced aneurysm, drugs such as coumarins are recommended for greater inhibition of blood clotting. If necessary, cardiac surgery is indicated for Kawasaki syndrome. In addition, cortisone-like drugs are being explored in clinical trials, and at least a complementary beneficial effect has been demonstrated for the treatment of Kawasaki syndrome.After successful completion of therapy, Kawasaki syndrome should be followed by regular checkups with a cardiologist to rule out long-term cardiac impairment (eg, stenosis).
Outlook and prognosis
Kawasaki syndrome is a very serious infection that absolutely requires medical treatment to avoid complications and possible late effects. However, parents of affected children can help relieve symptoms with simple remedies. In most cases, cold calf compresses are sufficient to reduce fever. If these do not help and the fever is dangerously high, a shower can provide relief. Directly a cold shower or a cold full bath are to be avoided. The blood vessels in the extremities would quickly constrict and the heart would have to beat faster. This is too much of a strain on the already stressed circulatory system. Instead, if you regulate the temperature slowly and not too far down, this leads to a moderate reduction in body temperature and only minimal circulatory stress. Because children dehydrate very quickly, adequate hydration on the part of parents is essential. Patients must be slowed down in their physical activity to avoid possible cardiac damage and later complications. The simple addition of fresh or dried turmeric to food, thanks to its anti-inflammatory effect, helps with both fever and the skin rashes that occur, which can also be alleviated with creams. Regular cardiological control after the symptoms subside is advised, so that possible late effects can be treated early.
Prevention
Because of the unknown etiology (underlying cause) of Kawasaki syndrome, no preventive measures exist.
Follow-up
In most cases, there are no specific or direct follow-up options available to the affected person for Kawasaki syndrome in babies and children. However, with this condition, a doctor should be seen very early to prevent further complications and also to prevent further worsening of symptoms. Early diagnosis usually always has a very positive effect on the further course of the disease, so that affected parents should contact a doctor at the first symptoms and signs of Kawasaki syndrome in babies and children. The treatment itself is usually carried out by taking certain medications. Here, the parents should pay attention to a correct dosage and also to a regular intake of the medication. In case of any uncertainties or questions, a doctor should always be consulted first, so that no further complaints occur. Likewise, in the case of Kawasaki syndrome in babies and children, regular checks and examinations by a doctor are very important in order to permanently monitor the condition of the affected person. At the same time, the child should not exert himself unnecessarily, since the heart is generally weakened. In many cases, this disease reduces the life expectancy of the affected person.
What you can do yourself
Kawasaki syndrome is a serious infectious disease in young children. Medical treatment is necessary to avoid complications and late effects. Nevertheless, parents can alleviate the symptoms by some means. The disease is accompanied by high fever. Calf compresses or moderate whole-body cooling usually lower the body temperature by one to two degrees. Because of the fever, parents should also make sure that their sick child drinks enough fluids. Still mineral waters or unsweetened herbal teas are ideal. To avoid complications of the heart, strict physical rest should be observed. For the treatment of skin rashes, cooling compresses are recommended, as well as the application of ointments with an anti-inflammatory effect. Coconut oil – which also has an antiseptic effect – or olive oil are recommended as natural substances. Special preparations can also be purchased in pharmacies. Naturopathy recommends the homeopathic remedy Belladonna for high fever. Further to the administration of Vitamin C preparations one advises, since with an inflammatory process this vital Vitamin is increased used up. Turmeric root also has a strong anti-inflammatory effect. Parents can buy this fresh in the supermarket or in powder or capsule form (dietary supplements) in the pharmacy.After surviving an illness, parents should take their child for regular checkups with a cardiologist to promptly detect and treat the occurrence of late effects.
