- A. Jacob
Summary of the
The Kawasaki syndrome is one of the last paediatric diseases, the exact etiology of which is still unclear with probably infectious genesis. There is a genetic predisposition; the incidence varies according to ethnicity. The clinical picture is well described, but the similarity to other diseases and the lack of “classical criteria”, the so-called incomplete Kawasaki syndrome, complicate the diagnosis. Further symptoms, certain laboratory constellations and echocardiography can help to initiate timely therapy and also reduce cardiac effects. Intravenous immunoglobulins have a proven effect in this respect. Alternatives are available, especially for therapy refraction. In the acute stage, impairment of the entire heart can occur as pancreatitis. Long-term morbidity is determined by the development of coronary aneurysms. It is true that with an increase in the coronary aneurysm, the tendency to regression decreases and the risk of thrombosis and the development of stenoses increases. Accordingly, long-term child cardiological care is sometimes necessary for the rest of one’s life.
R. Berner, Dresden
B. Koletzko, Munich
W. Sperl, Salzburg
An erratum to this post can be found at http://dx.doi.org/10.1007/s00112-016-0107-x .
The Kawasaki disease is one of the last pediatric diseases whose precise etiology is still unclear at probably infectiological genesis. A genetic predisposition exists, as the incidence varies depending on ethnicity. The clinical picture is well described, but the similarity to other diseases and lack of “classic criteria”, so-called “Incomplete Kawasaki disease” complicate the diagnosis. Other symptoms, certain laboratory constellations and echocardiography can help to initiate timely therapy and thereover reduce the cardiac involvement. Intravenous immunoglobulins have a proven effect in this regard. Alternatives, especially for refractory cases are available. In the acute stage, the cardiac involvemnet may present as pancarditis. The long-term morbidity though is determined by the development of coronary aneurysms. With increasing aneurysms decreases the trend of regression and increases the risk of thrombosis and development of stenosis. Therefore lifelong cardiological long-term follow-up may sometimes be necessary.
Special thanks to Mr. PD. Dr. M. Hufnagel for important support and discussions.
Compliance with ethical guidelines
conflict of interest
A. Jakob states that there is no conflict of interest.
This contribution does not include studies on humans or animals.
Which statement on the cause of the KS is correct?
A genetic predisposition is not assumed in KS.
Toxins play a key role in the development of KS.
Immunoglobulin A plasma cells in the respiratory tract could be an indication of viral genesis.
The antibody profile of patient sera clearly demonstrates certain Candida subspecies as a trigger for KS.
T cells do not seem to play a role in the pathogenesis of KS.
Which of the following symptoms would you most likely expect in a patient with KS?
Vesicular exanthema spitting out the inguinal region
Whitish stained coatings of the tongue
Unilateral exudative conjunctivitis
Which of the following symptoms does not usually help you to recognize KS early?
Bilateral nonexudative conjunctivitis
Polymorphic, truncal exanthema
Skin scaling on fingers and toes
Cervical lymphadenopathy (lymph nodes > 1.5 cm)
reddening of mouth and throat mucosa
A 5-month-old infant with 7 days of rhinitis and cough with fever up to 39.5 °C is presented. During the clinical examination you will notice a pale pink, maculopapular, confluent exanthema, especially on the trunk; the mucous membranes, including lips and tongue, are very dry and reddened. They feel an enlarged liver. Otherwise, no pathological findings can be found. Which statement do you consider to be correct?
Scarlet fever would not be considered for differential diagnosis in this patient.
This patient has a complete KS.
The fever is too high for a “viral airway infection”.
A systemic form of juvenile idiopathic arthritis may present with the same symptoms.
The fever focus must be bacterial.
Which of the following laboratory parameters would you most likely expect if you suspect KS?
Restricted glomerular filtration rate
You see from the clinical symptoms and the laboratory constellation that your patient should be treated like a KS. Which statement is correct in this respect?
At about 10-15 % the standard therapy shows no effect.
Echocardiography must be performed before the start of treatment.
High-dose ASS (30-50 mg/kgKG daily) reduces the rate of coronary aneurysms.
Intravenous immunoglobulins have the best effect when given spread over several days.
Steroids are contraindicated at KS.
Which cardiac findings in a 3-year-old patient are most likely to indicate Kawasaki syndrome as the cause of fever?
Pronounced vegetation on the mitral valve
Right ventricular pressure increase sign
An isolated, haemodynamically relevant pericardial effusion
Which acute therapy do you think is most indicated for KS?
Methylprednisolone (30 mg/kgKG daily) for 3 days
Immediate administration of IVIG 2 g/kgKG in one dose and ASS 3-5 mg/kgKG daily
Only IVIG 1 g/kgKG on 2 days
Only ASS 30-50 mg/kgKG daily
Infliximab 5 mg/kgKG daily
In a 5-year-old patient with KS, echocardiographically all coronary arteries up to a few centimetres after leaving the aortic root can be visualized without dilatation 2 weeks after the disease. The distal sections were not visible. What is the most appropriate procedure?
Further echocardiographic follow-ups are not necessary in this patient.
A further control appointment 6 to 8 weeks after the onset of the disease is indicated.
Coronary angiography should be performed.
CT angiography is indicated 6 weeks after the disease.
Acetylsalicylic acid in low doses can now be discontinued.
Which statements on long-term pharmacotherapy of KS apply?
Acetylsalicylic acid and Marcumar may be indicated.
Acetylsalicylic acid (3-5 mg/kgKG daily) should be given to each patient for at least one year.
Acetylsalicylic acid (3-5 mg/kgKG daily) can always be discontinued after 8 weeks.
Acetylsalicylic acid should preferably be given in combination with Plavix®.
Only patients with proven coronary aneurysms should receive ASS (3-5 mg/kgKG daily).