Kawasaki syndrome

Kawasaki syndrome

Kawasaki syndrome– acute febrile illness of childhood, characterized by damage to the coronary and other vessels: their dilatation, the formation of aneurysms, thrombosis and ruptures of the vascular wall are possible. Incidence rates are highest in Japan (highest incidence of HLA-Bw22 Ag in the population); in Europe and Israel (HLA-Bw51). In siblings, the incidence is somewhat higher than in the general population. The predominant age is 1-5 years. Etiology. The main hypothesis: staphylococcal and streptococcal superantigens stimulate the population of T-lymphocytes, which leads to the development of immune responses to antigen in endothelial cells. Risk factors. Environmental pollution: prolonged contact with detergents for washing carpets and living near water bodies. Pathomorphology. In the acute phase, neutrophilic infiltration of the pericardium, myocardium, endocardium and vascular endothelium. Vasculitis develops with damage to internal organs, necrosis, leading to aneurysmal dilatation of medium-sized arteries. In the second week of the disease, mononuclear infiltration prevails, gradually disappearing with or without the development of fibrosis.

Clinical picture

    • Fever lasting 2-3 weeks
    • Skin syndrome
    • Polymorphic rash – spotted, scarlet-like, morbilliform, erythematous; not often observed vesiculo-pustular rash
    • Rash on the skin of the perineum, followed by peeling
    • Redness of the palms and soles on the 3rd-5th day of illness. Peeling of the fingertips in the convalescence phase
    • Dense edema of the hands and feet on the 4th-7th day of illness
    • The defeat of the conjunctiva without exudation and suppuration.
    • Damage to the lips and oral mucosa
    • Cracks, sores on the lips
    • strawberry tongue
    • The mucous membrane of the mouth and pharynx diffusely injected
    • Cervical lymphadenopathy – lymph nodes are dense, sometimes painful
    • Defeat SSS
    • Tachycardia inappropriate to the degree of increase in body temperature
    • Myocarditis
    • Pericarditis, often subclinical
    • Aneurysms of the coronary and other medium-sized arteries
    • Gastrointestinal lesion
    • Diarrhea
    • Non-calculous cholecystitis
    • Pancreatitis
    • Damage to the urinary system
    • Glomerulonephritis
    • Urethritis
    • Lung damage
    • Pneumonitis
    • Atelectasis
    • Pleurisy
    • Joint damage
    • Arthralgia
    • Arthritis with damage to the wrist, knee and ankle joints for 3 weeks

nia

    • Damage to the nervous system
    • Increased excitability, disproportionate to the degree of increase in body temperature
    • Aseptic meningitis
    • Peripheral neuropathy.

Criteria of the classical flow. Fever for 5 days or more with 4 of 5 of the following:

    • Mucosal changes
    • Changes in the skin of the limbs or joints
    • Cervical lymphadenopathy
    • Rash
    • Conjunctival injury. Laboratory research
    • Anemia (normochromic normocytic)
    • Leukocytosis (12-40×108/l)
    • Thrombocytosis up to 750-1500x10u/l
    • Slight increase in bilirubin and transaminase levels
    • Ple-

cytosis in the CSF.

Special Studies

    • ECG – arrhythmias, myocardial ischemia
    • Echocardiography — cardiomyopathy, pericarditis.

Drug therapy

    • Antibiotics are used until a bacterial etiology of the disease is excluded.
    • Aspirin (acetylsalicylic acid) 80-150 mg/kg/day in 4 doses throughout the febrile period to reduce the likelihood of coronary artery disease. After normalization of body temperature, it is recommended to continue taking the product at doses of 5–10 mg/kg 1 r/day as an antiplatelet agent for several months (depending on the course of the disease). Periodic general blood tests, urine tests, liver function tests, ECG are required
    • Immunoglobulin 400 mg/kg/day IV for 4 days or 2 g/kg as a single dose (in combination with aspirin) when used in the first 10 days of illness reduces the risk of aneurysm formation and may reduce the duration of the acute phase
    • Dipyridamole (4 mg / kg / day) – with damage to the coronary vessels
    • Fibrinolytic agents – for thrombosis of the coronary arteries
    • Prostaglandin E to resolve ischemia
    • Heparin with subsequent replacement with indirect anticoagulants – for large or multiple aneurysms
    • Glucocorticoids can cause an increase in the incidence of aneurysms, so they are used only when absolutely necessary.

Complications

    • Coronary artery aneurysms – at the stage of convalescence in 20% of untreated patients
    • The development of coronary artery disease in children with risk factors:
    • Aneurysms with a diameter of more than 8 mm
    • Disseminated or saccular aneurysms
    • Fever lasting more than 21 days
    • Glucocorticoid therapy
    • Age over 2 years.

Synonyms

    • Mucocutaneous lymphonodular syndrome
    • Children’s polyarteritis

See also Scarlet fever, Measles, HIV infection and AIDS, Infectious mononucleosis, Arthritis, rheumatoid disease. MSO.3 Mucocutaneous lymphonodular syndrome (Kawasaki)

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