From this article you will get all the necessary information about Kawasaki syndrome. The mechanism of development of pathology, the possible causes of its occurrence, symptoms, diagnostic methods and methods of treatment.
Causes of
Kawasaki disease is a rare serious disease characterized by inflammatory lesions of coronary and other arteries of different calibers. It develops mainly in children under 5 years, much less often in adults at the age of 20-30 years.1.5 times more often detected in boys.
This disease is also called a periarteritis nodosa of childhood, generalized vasculitis and mucocutaneous lymph node syndrome. It is dangerous for its complications, including the formation and rupture of aneurysms, the development of myocarditis, arthritis, aseptic meningitis, etc. Due to the development of heart defects in children, this pathology in developed countries has outstripped rheumatic fever.
The development mechanism is as follows. For as yet undefined reasons, the body begins to develop antibodies to its own endothelial cells, of which the walls of the vessels are composed. Due to immune reactions, pathological changes begin to occur in them:
- the median membrane - the media - becomes inflamed, its cells die;
- internal and external membranes are destroyed, leading to the formation of pathological expansions in the walls - an aneurysm.
Without treatment after 1-2 months, fibrosis of the vascular walls begins, the arteries narrow, and in severe cases they completely close, the "closure" of the vessels develops.
When the adequate therapy is started, the prognosis is more often favorable, but in some cases the risk of death( up to 3% of the total number of cases) is possible because of thrombosis of the arteries or acute myocardial infarction.
Kawasaki syndrome refers to rheumatological, so its treatment is engaged in a rheumatologist. Depending on the complexity of the situation, a cardiologist or cardiac surgeon can be connected to the treatment.
Causes of Kawasaki syndrome development
Rheumatology to date does not have unambiguous precise data on the causes of inflammation of the vessel walls. There are several theories of the development of the syndrome. The most recognized is the hereditary predisposition, coupled with the effect on the organism of microorganisms of a viral and bacterial nature - the Epstein-Barr virus, parvovirus, rickettsia, herpes simplex virus, spirochete, retrovirus, streptococcus, staphylococci. Up to 10% of people whose ancestors suffered Kawasaki's disease also get it.
Prerequisites for the development of the syndrome:
- Race. Propensity to the disease is found in Asians.
- Reduced protective immunity functions.
Symptoms of the disease
There are 3 periods of generalized vasculitis:
- Acute. The first 7-10 days last.
- Subacute with a duration of 2-3 weeks.
- Period of convalescence( recovery).Takes from several months to 2 years.
Kawasaki syndrome in children begins violently and violently. The temperature rises to 39.0-40.0 degrees, is intermittent, persists for the first 5-7 days, and without treatment - up to 2 weeks. An increase in the duration of the febrile period is a bad sign for further prognosis. Against the background of febrile temperature, regional( more often cervical) lymph nodes increase, symptoms of severe intoxication are added - strong weakness, increased heart rate, abdominal pain, nausea, diarrhea. The child is suffering a serious illness, becoming irritable, restless. He often cries, refuses food, does not sleep well.
Skin manifestations develop during the first 5 weeks of the disease. On the body appear small blisters, scarlet fever or korepobodnaya rash. Its elements are located on the limbs, the body, in the groin. Gradually, on the feet and palms appear reddened areas, the skin becomes denser, starts to ache, because of what the movements of the fingers are limited. The feet swell. Eruption lasts 7 days after the appearance, and erythema persists up to 3 weeks, after which the skin begins to peel.
The defeat of the mucous in the acute period is manifested in the form of conjunctivitis of both eyes. In some, anterior uveitis - an inflammation of several elements of the choroid of the eyes - is attached to it. The mucous mouth turns red, becomes dry, the lips become covered with painful bleeding cracks, the tonsils grow, the color of the tongue changes to crimson.
Symptoms of Kawasaki syndrome: eye sprays, spalling and condensation of the skin of the feet, crimson color of the tongue, enlarged cervical lymph nodes Changes in the vessels and heart in Kawasaki disease in children lead to the development of myocarditis with pain, tachycardia or arrhythmia, dyspnea, often complicated by acute insufficiencyheart. The pericardium pouch - pericardium - is less often inflamed and mitral or aortic insufficiency is formed. In 25% of patients at 5-7 weeks, enlargement of individual sections of the walls of the coronary vessels occurs. Aneurysms of the subclavian, ulnar or hip arteries are not excluded.
Joint damage is detected in approximately 35% of patients who have both small and large joints with the corresponding symptoms.
Inflammation of the coronary arteries in adults with Kawasaki syndrome leads to loss of their elasticity and multiple aneurysms( enlargements), which increases the risk of developing dystrophy or myocardial infarction, thrombosis, calcification and early atherosclerosis. Patients are concerned about pain in the heart and joints, violation of heart rate and digestion. In some cases, it is possible to develop meningitis, urethritis, and damage to the organs of the digestive tract.
The patient has problems with the exercise of the usual physical activity. Some daily activities, such as climbing a mountain, fast walking or running, lifting weights, become difficult.
After treatment, abnormal vascular wall dilatations in adults remain, but eventually decrease and can disappear completely.
Diagnosis of Kawasaki syndrome
Kawasaki's disease is confirmed by the presence of a minimum of 4 clinical and diagnostic criteria against the background of a 5-day fever. Among them:
- Conjunctivitis of both eyes.
- Local( cervical) adenopathy.
- Polymorphic rash all over the body.
- Symptoms of damage to the oral mucosa.
- Redness, condensation of the skin of the feet and palms with their swelling.
- Peeling of the pads of the fingers for 2-3 weeks from the onset of the disease.
If an aneurysm of the coronary arteries is detected during the examination, 3 additional signs are sufficient to establish the diagnosis.
Click on photo to enlarge Atypical( incomplete) Kawasaki syndrome is diagnosed if there are 2-3 of the above criteria. This is because the symptoms can be caused by another pathology. For a complete clinical picture of the disease is not enough in the collection of objective data.
diagnostic methods
| Laboratory studies | Instrumental investigations |
|---|---|
| CBC | ECG |
| «Biochemistry» Blood | ultrasound |
| heart Urinalysis | X-ray of the chest |
| The study of the cerebrospinal fluid | angiography of the coronary arteries |
It is important to make a differential diagnosis of influenza syndromeStevens-Jones, rubella, measles, scarlet fever, mycoplasmal pneumonia, rheumatoid arthritis, adenovirus infection, mononucleozom.
Multislice computed tomography( MSCT) of coronary vessels, 3D-reconstruction. Aneurysms are indicated by arrows Treatment of Kawasaki syndrome
This disease is well treatable and completely cured, although there are cases of death due to the development of serious complications, for example, myocardial infarction. The earlier the Kawasaki disease is diagnosed and the treatment is started, the more favorable the prognosis.
Because the exact causes of the pathology are not established, treatment aimed at eliminating the cause is not provided. To relieve the symptoms and prevent the further development of inflammation apply the drugs from the table below.
| Name of the medicinal product or pharmacological group | Action, use |
|---|---|
| Immunoglobulin | The main drug. Enter in / in-drip for 9-12 hours every day. The best effect is achieved when it is introduced in the first 10 days of the disease. Reduces inflammation in the walls of the arteries. |
| Acetylsalicylic acid | Large doses are prescribed during the febrile period( 5 days) with a decrease in dosage to preventive in the next 2-3 months. Aspirin dilutes blood, relieves inflammation, reduces the risk of blood clots. |
| Anticoagulants - clopidogrel, warfarin | Recommended for children with an aneurysm revealed in order to prevent thrombosis. |
Children must be vaccinated against chicken pox, influenza, because long-term admission in large doses of aspirin along with these infections increases the risk of developing acute hepatic encephalopathy - Ray's syndrome.
The administration of corticosteroids is controversial. It is proved that hormones increase the risk of aneurysm and coronary thrombosis.
Complications of
- From the heart and blood vessels: myocarditis, damage to the heart valves, aneurysms of the arteries, myocardial infarction.
- From other organs: diarrhea, aseptic meningitis, soft tissue gangrene, arthritis, cholecystitis with obstruction of passage of bile through the ducts, otitis, etc.
Gangrene of the finger Forecast for Kawasaki disease
Despite the likelihood of complications, the prognosis for this pathology is favorable. With timely treatment begun, recovery occurs in 6-10 weeks. Death ends only in 0.8-3% of neglected cases, when therapy was not started on time;death comes from a heart attack, thrombosis, less often - myocarditis with severe heart failure.
