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 mainly found in Japan and with increasing frequency in Germany (about 9 out of 100,000 children).
What is Kawasaki Syndrome?
Infants and young children suffering from Kawasaki syndrome suffer from severe inflammation of the vessels throughout the body. The inflammation causes symptoms such as fever and a rash.
Kawasaki syndrome (also mucocutaneous lymph node syndrome) is an acute fever illness that primarily occurs in infancy, particularly between the ages of 2 and 5, and is associated with vasculitis, an inflammation of the small and medium-sized blood vessels. See AbbreviationFinder for abbreviations related to Kawasaki Syndrome.
In most cases, the Kawasaki syndrome, which is initially very similar to other infectious diseases such as scarlet fever or measles, also manifests itself as a systemic inflammation of the internal organs.
In addition to the suddenly occurring fever, there are swellings in the lymph nodes in the neck and throat (cervical lymphadenopathy), non-purulent conjunctivitis (conjunctivitis), stomatitis (inflammation of the oral mucosa) with strawberry or lacquer tongue, a polymorphic exanthema (non-itching, multi-shaped 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 clarified.
It is assumed that the etiology of Kawasaki syndrome is due to an infection with previously unidentified bacteria or viruses whose superantigens (toxic proteins of the pathogens) cause the affected children’s immune system to react with pronounced inflammatory reactions as a result of a genetic defect.
The significantly higher incidence (number of new cases) of Kawasaki syndrome in Japan compared to Europe or North America also speaks for a genetic predisposition (predisposition).
Symptoms, Ailments & Signs
Infants and young children suffering from Kawasaki syndrome suffer from severe inflammation of the vessels throughout the body. The inflammation causes symptoms such as fever and a rash. The disease is characterized by a gradual rise in body temperature, which is usually associated with chills, weakness and a general feeling of being unwell.
The rash presents as painful redness that can appear on any part of the body and occasionally develops into ulcers or cysts. It can also lead to conjunctivitis and swelling of the mucous membranes in the mouth and throat. Also characteristic are the red lips and the reddish tongue, also strawberry tongue. If treated early, affected children usually recover relatively quickly.
In severe cases, however, the rash can leave scars or lead to permanent sensory disturbances. If not treated, there is a risk of death. Then the skin lesions worsen and the fever increases. Eventually, there may be complications of the heart and the death of the child as a result. The symptoms usually appear between the ages of one and eight. However, adolescents and young adults can also be affected. Kawasaki syndrome is particularly common in boys.
Diagnosis & History
Kawasaki syndrome can be diagnosed based on the characteristic symptoms. In addition to the acutely occurring high fever, at least four of the main symptoms of the disease must be detectable in order to assume Kawasaki syndrome.
In addition, similar infectious diseases such as measles or scarlet fever should be excluded in the differential diagnosis. The diagnosis is confirmed by a blood analysis. So-called autoantibodies (cANCA), antibodies directed against endogenous antigens, can be serologically detected in Kawasaki syndrome. An increased number of leukocytes (white blood cells) and an increased CRP value (C-reactive protein) also indicate a possible Kawasaki syndrome as inflammatory markers.
In order to rule out involvement of the coronary vessels (arterial coronary vessels) or a heart aneurysm (protrusion of the arterial coronary vessels), an echocardiography (heart ultrasound) should be performed.
If left untreated, Kawasaki syndrome can lead to an aneurysm of the heart, which can lead to life-threatening secondary diseases such as myocarditis (inflammation of the heart muscle), infarcts, pericarditis (inflammation of the heart sac) and cardiac arrhythmias, which have a significant impact on long-term mortality (mortality rate). With timely diagnosis and treatment, the prognosis is good and the disease usually heals without complications.
Complications
In most cases, the Kawasaki syndrome in babies and children leads to a very strong fever. Those affected also suffer from a red discoloration of the skin, lips and tongue. The mucous membranes in the throat and mouth also swell, making it difficult to eat and drink.
It is not uncommon for babies and children to have Kawasaki syndrome with a rash all over the body and inflammation of the conjunctiva. The patient’s quality of life is significantly restricted and reduced by this syndrome. If left untreated, the syndrome can also cause heart problems and inflammation, which can eventually lead to death.
The treatment itself usually does not lead to any particular complications and the course of the disease is always positive. This is done with the help of medication that significantly reduces the discomfort and symptoms. If the treatment is successful, there are no further complications or reduced life expectancy. Consequential damage does not occur with successful treatment of Kawasaki syndrome in babies and children.
When should you go to the doctor?
A doctor’s visit is necessary if the fever persists or increases. Redness of the skin, an increase in cardiac activity and changes in the complexion must be examined and examined by a doctor. Children up to the age of 5 who suffer from sudden acute symptoms that lead to serious health impairments must be treated by a doctor as soon as possible. Since Kawasaki syndrome in babies and children can lead to premature death in severe cases without medical care, a doctor’s visit is recommended at the first irregularities.
Red discoloration of the lips and tongue resembling strawberry color should be evaluated. Swelling of the lymph, pain in the lymph nodes or a change in the mucous membranes in the mouth and throat should be presented to a doctor. If conjunctivitis occurs, you need to see a doctor. In the event of cardiac arrhythmias, a doctor must be consulted immediately. The heart muscle can be damaged, which can have serious consequences. If the child refuses to eat and drink enough fluids, there is a risk that the organism will be undersupplied. A visit to the doctor is required without delay so that a life-threatening condition does not develop. If the child shows behavioral problems or an increased need for sleep, a doctor should be consulted.
Treatment & Therapy
In the case of Kawasaki syndrome, the therapeutic measures are primarily aimed at reducing the inflammatory reactions and avoiding aneurysms in the coronary arteries, for which there is an increased risk of manifesting in the second and third week. For this purpose, immunoglobulins, which play an important role in the body’s defenses and cannot be synthesized to a sufficient extent by the children affected by Kawasaki syndrome, are infused (2 g/kg body weight over 12 hours).
If necessary, the therapeutic measure must be repeated if the affected child does not respond sufficiently. In addition, high-dose acetylsalicylic acid (30 to 100 mg/kg body weight per day) is used to reduce acute inflammation and fever. As long as there is a risk of a coronary aneurysm, acetylsalicylic acid should continue to be taken at a lower dose (2 to 4 mg/kg body weight per day) because the substance has an anticoagulant effect.
If there is a pronounced aneurysm, drugs such as coumarins are recommended to inhibit blood clotting more. If necessary, cardiac surgery measures are indicated for Kawasaki syndrome. In addition, cortisone-like drugs are being researched as part of clinical tests, with at least one additional positive effect for the treatment of Kawasaki syndrome being demonstrated. After the successful end of therapy, regular check-ups by a cardiologist should take place in the case of Kawasaki syndrome in order to rule out long-term damage to the heart (e.g. stenoses).
Outlook & Forecast
Kawasaki syndrome is a very serious infection that requires medical treatment to avoid complications and possible long-term effects. However, parents of the affected children can help to alleviate the symptoms with simple means.
In most cases, cold calf wraps are sufficient to reduce the fever. If these do not help and the fever is dangerously high, a shower can provide relief. Direct cold showers or cold baths should be avoided. The blood vessels in the extremities would constrict quickly and the heart would have to beat faster. This is too much of a strain on the already stressed circuit. If you instead regulate the temperature slowly and not too far, this leads to a moderate drop in body temperature and only minimal strain on the circulatory system.
Because children dehydrate very quickly, it is imperative that parents stay hydrated. Patients have to be slowed down in their physical activity in order to avoid possible heart damage and subsequent complications. Simply adding fresh or dried turmeric to food, thanks to its anti-inflammatory effect, helps with both fever and skin rashes, which can also be alleviated with creams.
A regular cardiological check-up after the symptoms have subsided is advisable so that possible late effects can be treated at an early stage.
Prevention
Due to the unknown etiology (underlying cause) of Kawasaki syndrome, no preventive measures exist.
Aftercare
In most cases, those affected with Kawasaki syndrome in babies and children have no special or direct options for aftercare. With this disease, however, a doctor should be consulted very early on, so that there are no further complications and no further deterioration of the symptoms.
An early diagnosis usually always has a very positive effect on the further course of the disease, so that the parents concerned should contact a doctor as soon as the first symptoms and signs of Kawasaki syndrome appear in the baby and child. The treatment itself is usually carried out by taking certain medications.
Parents should ensure that the dosage is correct and that the medication is taken regularly. If anything is unclear or if you have any questions, you should always consult a doctor first so that no further problems arise. Regular check-ups and examinations by a doctor are also very important for babies and children with Kawasaki syndrome in order to monitor the condition of the affected person over the long term. The child should not exert himself unnecessarily, since the heart is generally weak. In many cases, this disease reduces the life expectancy of those affected.
You can do that yourself
Kawasaki syndrome is a serious infectious disease in young children. Medical treatment is necessary to avoid complications and long-term effects. However, there are a number of ways parents can reduce the symptoms.
The disease is accompanied by a high fever. Calf wraps or moderate full-body cooling usually lower the body temperature by one to two degrees. Due to the fever, parents should also ensure that their sick child is drinking enough fluids. Still mineral water or unsweetened herbal teas are ideal. In order to avoid complications in the heart, strict attention must be paid to physical protection. Cooling compresses and the application of anti-inflammatory ointments are recommended for treating the rash. Coconut oil – which also has an antiseptic effect – or olive oil are recommended as natural substances. Specially mixed preparations can also be purchased in pharmacies.
Naturopathy recommends the homeopathic remedy Belladonna for high fever. Furthermore, the administration of vitamin C preparations is recommended, as this vital vitamin is consumed at an increased rate during an inflammatory process. The turmeric root also has a strong anti-inflammatory effect. Parents can buy these fresh in the supermarket or in powder or capsule form (nutritional supplements) in the pharmacy.
After the illness has been overcome, parents should take their child to a cardiologist for regular check-ups in order to be able to promptly identify and treat the occurrence of long-term effects.