Kawasaki Disease (KD,Mucocutaneous Lymph Node Syndrome,)

Kawasaki Disease

What Is The Kawasaki Disease?

Kawasaki disease is a disease named after a Japanese doctor, also known as mucocutaneous lymph node syndrome, which is a pediatric disease with acute fever and rash as the main symptoms.

Fuji Kawasaki of Japan made the first report in 1967, thus named after him. Kawasaki disease can cause severe cardiovascular disease, which has attracted more and more attention from parents and doctors.

The incidence of Kawasaki disease is the highest among children living in East Asia or Asian children living in the rest of the world, with 264.8 per 100,000 children under 5 years of age in Japan in 2012. From 2003 to 2006, the incidence rate of Kawasaki disease in children under 5 years old was 69/100,000 in China (including Taiwan Province).


What Is The Cause Of Kawasaki Disease?

The etiology of Kawasaki disease is still unclear. Possible causes are as follows:

  • Immune response: Kawasaki disease is a systemic inflammatory disease that often involves medium-sized arteries, especially the coronary arteries. Plasma cells producing oligoclonal IgA antibodies were found in the arteries and respiratory tract of children with Kawasaki disease, suggesting a possible immune response.
  • Infectious etiology: According to the seasonality and prevalence of Kawasaki disease, it is suggested that Kawasaki disease is related to infection, but the etiology remains unclear.
  • Genetic factors: The high incidence of Kawasaki disease among Asian and Asian-American individuals and family members with indicated cases suggests that genetic factors may also play a role in its pathogenesis.
  • Environmental factors: There was a certain correlation between Kawasaki disease and mercury poisoning and atopic dermatitis, so it was deduced that Kawasaki disease was related to factors that could cause dermatitis, such as mercury and dust mite in the environment.

What Are The Symptoms Of Kawasaki Disease?

What are the manifestations of Kawasaki disease?

The lesion of Kawasaki disease is vasculitis. As blood vessels are distributed all over various organ systems of the whole body, Kawasaki disease has the manifestation of multiple organ systems. Among which is helpful for the diagnosis, parents need to pay attention to the following symptoms:

  • Fever of unknown origin, lasting 5 days or more.
  • Bilateral conjunctival congestion (red eyeball).
  • The oral cavity and pharyngeal mucosa were suffused with blood and redness, with red and chapped lips, and red and swollen tongue surface like waxberry.
  • In the early stage of disease, hands and feet were hard and swollen, palms and soles were red, and the tips of fingers and toes peeled off during the disease stage.
  • Erythema and irregular shape appear on the anterior chest and back. It is necessary to pay attention to whether there are blisters and scabs.
  • Cervical lymph node swelling.

What are the possible complications of Kawasaki disease?

Children with kawasaki disease in addition to some typical symptoms, there are other some performance:

  • Nervous system: irritability, irritability, and a few meningitis manifestations such as convulsions and coma can occur.
  • Digestive system: abdominal pain, nausea, diarrhea, abdominal distension, decreased stool, large liver, jaundice, etc.
  • Cardiovascular system: various heart diseases such as pericarditis, myocarditis, endocarditis, arrhythmia, coronary artery dilatation, coronary aneurysm, coronary artery thrombosis and even myocardial infarction can occur within 1–6 weeks of the onset.
  • Other: there may also be coughing, joint pain, arthritis, etc.

Why does coronary artery aneurysm occur in Kawasaki disease?

Coronary arteries are most affected by vascular inflammation caused by Kawasaki disease. When inflammation occurs in blood vessels, it may cause the vessel wall to become thinner. When blood flows through the thinner vessel wall, under the action of pressure, like blowing a balloon, a small bag will bulge out from the thinner part. This bulge-out bag is called coronary aneurysm. The coronary artery is the blood vessel that nourishes the heart. If the coronary artery is abnormal, the function of the heart may be affected.

Which children with Kawasaki disease prone to coronary artery aneurysm?

Coronary aneurysms may occur in children with the following characteristics.

  • Boy.
  • Less than 6 months or greater than 3 years of age.
  • Fever lasts for more than 2 weeks or recurs.
  • Cardiac enlargement with arrhythmia.
  • Recurrent kawasaki disease.
  • White blood cells, platelets, and erythrocyte sedimentation rate increased significantly, or continued unabated.

How To Check For Kawasaki Disease?

What check should kawasaki disease do?

Children suspected of Kawasaki disease need routine examinations such as routine blood tests, CRP, erythrocyte sedimentation rate, color Doppler echocardiography and electrocardiogram. The significance of these examinations is to avoid missed diagnosis and misdiagnosis, help early detection and early treatment, and reduce the possibility of serious complications of Kawasaki disease.

Why does Kawasaki disease need a heart color Doppler ultrasound examination?

The clinical manifestations of many children with Kawasaki disease are less typical, but the diagnosis of atypical Kawasaki disease can be made by echocardiography with typical lesions. Color Dopplar ultrasound of the heart can detect heart lesions in time to help diagnosis and timely treatment. Therefore, once Kawasaki disease is suspected, echocardiography (color Dopplar ultrasound) and other laboratory tests should be performed as soon as possible.

How to establish the diagnosis of kawasaki disease.

The diagnostic criteria for Kawasaki disease are as follows: Fever ≥ 5 days, and a diagnosis can be made with the presence of four of the following 5 criteria:

  • Bilateral conjunctival congestion (no exudation).
  • Changes in lips, tongue and oral mucosa (congestion, dryness, chapped, waxberry tongue).
  • Changes in the peripheral extremities (edema, erythema, peeling).
  • Erythema multiforme of the skin of the trunk.
  • Cervical lymph node enlargement (at least one lymph node ≥ 1.5 cm in diameter).

What is incomplete kawasaki disease?

Children with fever ≥ 5 days and two or three main clinical features other than exudative conjunctivitis, exudative pharyngitis, ulcerative stomatitis, bullous or vesicular rash, generalized lymphadenopathy or splenomegaly need to consider the possibility of incomplete KD. The possibility of incomplete KD needs to be considered in infants < 1 year of age who have been febrile for ≥ 7 days without any other explanation.

What diseases should be differentiated from Kawasaki disease?

Kawasaki disease needs to be distinguished from scarlet fever, staphylococcal epidermolysis syndrome, measles, drug fever, and juvenile rheumatoid arthritis.


How To Prevent Kawasaki Disease?

Can Kawasaki disease be prevented? How to prevent recurrence of kawasaki disease?

Since the pathogenesis of Kawasaki disease has not been fully investigated yet, most experts believe it is vascular inflammation caused by abnormal immune response, and every child's immune function is completely different. Therefore, there is no exact method to prevent Kawasaki disease. Kawasaki disease has a recurrence rate of 1%–2%, and there is no way to prevent recurrence.


How To Treat Kawasaki Disease?

Which clinic should I visit for Kawasaki disease?

Most patients with Kawasaki disease are children, so they often go to the pediatric clinic. After Kawasaki disease is considered, the patient is usually transferred to pediatric cardiology for further treatment.

What is the most effective treatment of Kawasaki disease?

Any child diagnosed with Kawasaki disease or incomplete Kawasaki disease should be treated at the time of diagnosis with intravenous immunoglobulin (IVIg) in combination with aspirin. Furthermore, IVIG is most effective when given within the first 7–10 days from the date of onset. However, when there is evidence of persistent vasculitis or systemic inflammation, such as persistent fever or increased reactants in the acute phase, IVIG should be used even if the course of the disease exceeds the window of 10 days.

Why should kawasaki disease patient take aspirin orally?

Kawasaki disease in the recovery period will appear thrombocytosis, 10 times or even dozens of times higher than normal, the possibility of thrombosis. Therefore, oral antiplatelet drugs are needed to prevent thrombosis.

Why do children with Kawasaki disease need a large dose of oral aspirin?

High-dose aspirin can quickly control the inflammatory reaction and prevent the occurrence of aneurysm.

Why does kawasaki disease use large dose gamma globulin to treat?

This is related to the pathogenesis of Kawasaki disease. At present, it is considered that Kawasaki disease is an abnormal immune response that causes inflammation and damage to human blood vessels. High-dose gamma globulin can inhibit this immune response, so as to achieve the purpose of controlling inflammation and treating diseases.

Under what conditions does Kawasaki disease need to be treated with glucocorticoids?

If an increased risk of IVIG resistance in children with Kawasaki disease is determined, it is recommended that glucocorticoids be added to the initial IVIG treatment. Prednisone/prednisolone was usually administered for a total of 15 days, initially as an intravenous dosage form, and orally 12 to 24 hours prior to expected discharge.

Glucocorticoid therapy may also be administered to non-Japanese pediatric patients who meet the following criteria: coronary artery enlargement, ≤ 12 months of age (especially < 6 months), Kawasaki disease with shock, and Kawasaki disease with macrophage activation syndrome (MAS) prior to IVIG treatment.

Are there any side effects of gamma globulin for Kawasaki disease?

  • Allergic reaction: Individual children may have an allergic reaction after receiving immunoglobulin. During infusion, parents should always pay attention to the changes of children and report any abnormality to health care personnel in time.
  • Side effects of the blood system: because gamma globulin contains a small amount of anti-red blood cell antibodies, it can cause hemolysis when used by individuals and requires urgent treatment.
  • Virus transmission: gamma globulin is a blood product. Generally, before application, a blood transfusion consent form is signed to inform parents of possible blood-borne diseases such as hepatitis B and AIDS. But the probability of this happening is very low.

What to do if coronary hemangioma occurs in Kawasaki disease? Can it heal itself?

Only a few coronary aneurysms die out on their own. In patients with single coronary aneurysms, long-term aspirin should be used until the aneurysm disappears. Long-term oral administration of aspirin and dipyridamole should be administered to patients with multiple or larger coronary aneurysms.

Children with larger aneurysms, which are prone to thrombosis and coronary artery stenosis or occlusion, require warfarin and monitoring of clotting time. These children can't take part in sports, limit the amount of activity appropriately. Cardiac-related tests are performed regularly. Thrombolytic therapy is needed for children with myocardial infarction and thrombosis.

Can kawasaki disease cure?

Kawasaki disease can be mostly cured. However, about 20%–30% of untreated children develop coronary artery aneurysm. After high-dose gamma globulin treatment, coronary artery lesions occur in about 15% of children. It is possible that 1%–2% children with Kawasaki disease may relapse.

How long can kawasaki disease cure commonly?

For the children with standardized treatment, they need to be hospitalized for about 5–7 days after the diagnosis is made. When the temperature returns to normal and the blood test results improve, they can be discharged to continue oral medication, and blood and cardiac color Doppler ultrasonography need to be regularly reviewed. The course of the disease varies according to the severity of the illness.

  • The first stage is acute fever, with a general course of 1–11 days.
  • The second stage was subacute, with a general course of 11–21 days, with decreased body temperature, remission of symptoms, membranous desquamation and thrombocytosis. Coronary aneurysm may occur, causing myocardial infarction and aneurysm rupture.
  • The third stage is the recovery period lasting from 21 to 60 weeks. If there is no obvious coronary aneurysm, the disease will gradually recover, and even if there is a coronary aneurysm, the disease can still develop sustainably.

What should pay attention to if children with Kawasaki disease using gammaglobulin?

When the child is discharged from hospital, the parents should confirm with the doctor whether the child has used gamma globulin, which can affect the child's immune system for a period of time. Within 11 months after using this medicine, it is not appropriate to vaccinate with live attenuated vaccine. The parents need to ask the staff when vaccinating.

What should pay attention to after the treatment of Kawasaki disease?

Parents should pay attention to regular reexamination after discharge. If there is no coronary artery disease, a comprehensive examination (including physical examination, ECG and echocardiography) can be conducted once one month, three months, half a year and one year after discharge.

For children with coronary artery disease, follow-up every 6 months until the coronary artery dilatation and coronary artery aneurysm disappear in addition to the above examination.


What Should Patients With Kawasaki Disease Pay Attention To In Life?

What should be noticed about the diet of Kawasaki disease patients during hospitalization?

During hospitalization, children with Kawasaki disease often receive intravenous immunoglobulin and high-dose oral anti-inflammatory and anti-platelet drugs. The following points should be noticed in their diet:

  • Gamma globulin may cause allergic reactions, so it is recommended that children eat a light diet, do not eat a special high protein diet, in case of food allergies to cover up static drops of gamma globulin allergic reactions.
  • Don't eat greasy food, in case of hyperlipidemia aggravate vascular damage.
  • High-dose aspirin may cause gastrointestinal bleeding, so it's best to eat soft food.

What does Kawasaki disease patient need to notice during taking medicine at home? What happens to need to go to the hospital?

Children with Kawasaki disease are usually prevented from thrombosis by oral medication at home, but it is also possible to cause bleeding. Generally pay attention to the presence of bleeding, including nose bleeding, stool bleeding, skin bleeding point, etc. If bleeding or fever occurs, go to the hospital in time for careful examination.

Kawasaki disease occurs in the first fetus, will the second child get Kawasaki disease?

Kawasaki disease is not a genetic disease. If the first child has Kawasaki disease, the probability of having Kawasaki disease in the second child is not higher than that in other children.

Can children with Kawasaki disease be vaccinated on time after receiving treatment?

  • For children who have been treated with IVIG, it is recommended to postpone vaccinations with live viruses such as measles and varicella vaccines for at least 11 months because IVIG can interfere with the immunogenicity of the vaccine.
  • Varicella vaccination is recommended for children ≥ 12 months of age who have been taking moderate or high doses of aspirin for a long period of time.
  • Influenza vaccination is recommended for all children over 6 months of age who require high-dose aspirin for a prolonged period of time.

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