Kawasaki disease

Introduction to Kawasaki disease Muco-cuta-meouslymphnodesyndrome (MCLS), also known as Kawasakidiseaes, is an acute febrile rash pediatric disease in which systemic vasculitis becomes the main pathology. In 1967, Japanese Kawasaki Fusuke doctor reported for the first time that serious cardiovascular disease can occur in this disease, which has attracted people's attention. In recent years, the incidence has increased. In the hospitalized cases of rheumatic diseases in Beijing Children's Hospital in 1990, 67 cases of Kawasaki disease and 27 cases of rheumatic fever; Among the same materials in 11 hospitals in other provinces and cities, Kawasaki disease is two parts of rheumatism. Obviously, Kawasaki disease has replaced rheumatic fever as one of the main causes of acquired heart disease in children in China. basic knowledge The proportion of illness: 0.04%-0.05% (the above is the incidence of children under 12 years old) Susceptible people: good for 6 to 18 months baby Mode of infection: non-infectious Complications: coronary aneurysm abdominal pain myocarditis

Cause of Kawasaki disease

The cause is not clear, the disease is a certain epidemic and landlord, clinical manifestations of fever, rash, etc., presumably related to infection, generally believed to be a variety of pathogens, including Epstein-Barr virus, retrovirus (retrovirus), or streptococci, Propionibacterium infection, reported in 1986, the peripheral blood lymphocyte culture supernatant increased the activity of reverse transcriptase, suggesting that the disease may be caused by retrovirus, but most studies have not obtained consistent results, and have previously proposed mycoplasma, Rickettsia, dust mite is the pathogen of this disease, and it has not been confirmed. Some people think that environmental pollution or chemical allergy may be the cause of the disease.

Recent studies have shown that the disease has obvious immune disorders in the acute phase, which plays an important role in the pathogenesis. In the acute phase, peripheral blood T cell subsets are imbalanced, CD4 is increased, CD8 is decreased, CD4/CD8 ratio is increased, and this change is in lesion 3~ The most obvious 5 weeks, return to normal after 8 weeks, the CD4/CD8 ratio increased, the body's immune system is activated, CD4 secreted lymphokines increased, promote B cell polyclonal water activation, proliferation and differentiation into plasma cells, leading to serum IgM , IgA, IgG, IgE is elevated, activated T cells secrete high concentrations of interleukins (1L-1, 4, 5, 6), r-interferon (IFN-r), tumor necrosis factor (TNF), these lymphocytes Factor, activin can induce endothelial cell expression and produce new antigen; on the other hand, it promotes B cell secretion of autoantibodies, which leads to cytotoxicity of endothelial cells, vascular inflammation caused by endothelial cell damage, 1L-11L-6, Increased TNF can induce hepatocytes to synthesize acute reactive proteins, such as C-reactive protein, r-antitrypsin, and haptoglobin, which cause acute fever in this disease. (CIC) increased, 50 to 70% of cases were not available in the first week of the disease, and reached the peak in the third to fourth week. The mechanism of action of CIC in this disease is still unclear, but there is no immune complex deposition in the lesion. The serum C3 does not decrease but rises, which is not in line with the general immune complex disease. The triggering cause of the above immune disorder is unknown. Today, it is believed that Kawasaki disease is an immune-mediated systemicity triggered by a host susceptible to various infectious agents. Vasculitis.

Kawasaki disease prevention

As for the extent to which the baby is good enough to go to a nursery school or kindergarten, there is no special rule. As long as the baby has no special discomfort, he can return to normal life. Generally speaking, Kawasaki disease will not recur in the future, but the heart disease is affected by the sequelae. It will appear after many years, so it is necessary to have a regular heart check.

Infants and children with this disease can be ill, but 80 to 85% of patients are within 5 years of age, and occur in infants between 6 and 18 months. There are more boys, males and females are 1.3 to 1.5:1, with no obvious seasonality. Or more summer, there were about 100,000 cases of Kawasaki disease in Japan until 1990, and three epidemics occurred in 1979, 1982, and 1986. The prevalence of children within 4 years of age was 172-194/100,000. Although the number of reports in the world is not as good as that in Japan, it is north to Sweden, the Netherlands, the United States, Canada, the United Kingdom, South Korea, South Africa, Australia, Singapore, etc., and China has first since Beijing, Shanghai, Hangzhou, and Rong in 1978. And Taiwan and other places reported a small number of cases, in 1989, "Practical Pediatrics" comprehensive 220 cases, the source throughout the country, 1983-1986 national major children's hospitals and medical schools affiliated hospitals communication survey, a total of 965 hospitalized cases, 1987 ~ 1991 In the second survey of the year, the number of hospitalized cases increased to 1969, and the annual increase trend, 78.1% of patients within 4 years old, male: 1.6:1 for women, more Japanese in the US cases, Japan reported sibling incidence 1 ~2%, suggesting genetic tilt .

The vast majority of children have a good prognosis, self-limiting, and appropriate treatment can gradually recover, which is very different from infant-type nodular arteritis, but 15 to 30% of patients with Kawasaki disease can develop coronary Aneurysms, due to coronary aneurysm, thromboembolism or myocarditis, accounted for 1-2% of all cases, even in the recovery period can be sudden death, the number of ischemic heart disease is very small, about 2% recurrence The mortality rate has dropped to 0.5% to 1.0% in recent years. The death causes of 104 cases of Kawasaki disease in Japan, 57% of myocardial infarction, 12% of heart failure, 6.7% of myocardial infarction with heart failure, 5% of coronary aneurysm rupture, arrhythmia 1 %, other patients have concurrent infections, Beijing Children's Hospital since 1986 applied two-dimensional echocardiography to examine 188 cases of Kawasaki disease, 60 cases of coronary artery disease were detected, including 44 cases of expansion, 16 cases of aneurysm, after follow-up for 3 months ~5 years, an average of 22.6 months, 40 cases of normal recovery, the latter 6 cases, the normal recovery time was 4.4 ± 2.9 months and 15.7 ± 17.2 months, 2 cases of death, 1 case of multiple moderate coronary aneurysms (inner diameter 7mm) Acute anterior myocardial infarction , And the other cases were multiple Juliu, cracking occurs in the course of the right coronary artery for 28 days.

Kawasaki disease complications Complications coronary aneurysm abdominal pain myocarditis

Because of cardiovascular disease, it is both the symptoms of the disease and the complications that can cause death. In this section, the history of the disease will be detailed, with an early detection and timely and appropriate treatment.

1, coronary artery lesions according to Japan's 1009 cases of Kawasaki disease observation, suggesting that transient coronary artery expansion accounted for 46%, coronary aneurysm accounted for 21%, the use of two-dimensional echocardiography found that coronary artery expansion can be on the third day of onset Appeared, most of them disappeared within 3 to 6 months, coronary aneurysms can be measured on the 6th day of onset, the highest rate was detected in the 2nd to 3rd week, and new lesions rarely appeared after the 4th week. The incidence of coronary aneurysms For 15 to 30%, the presence of clinical myocarditis does not predict coronary involvement, and some risk factors that are clearly associated with coronary aneurysms include age at onset within 1 year of age, boys, persistent fever over 14 days, anemia, total number of white blood cells at 30 Above ×109/L, the erythrocyte sedimentation rate exceeds 100mm/h, C-reactive protein is significantly elevated, plasma albumin is reduced and body aneurysms are produced. Most coronary aneurysms are self-limiting, and most of them resolve spontaneously within 1 to 2 years.

The coronary artery lesions of this disease are most common in the proximal end of the trunk, the left anterior descending artery, followed by the left circumflex artery, and the rare isolated distal aneurysm, which generally divides the severity of coronary artery disease into four degrees:

1 normal (0 degrees): no expansion of the coronary arteries,

2 mild (I degree): tumor expansion is obvious and limited, inner diameter <4mm,

3 moderate (II degree): can be single, multiple or extensive, inner diameter is 4 ~ 7mm,

4 severe (III degree): the internal diameter of the giant tumor is 8mm, mostly extensive, involving more than 1 branch, the incidence rate is about 5%, the prognosis is poor, so patients with coronary artery disease should be closely followed up to review the echocardiogram regularly, usually in The disease is checked once a week for 4 weeks, and then reviewed in February, half a year, and then reviewed at least once a year according to the degree of the disease. Coronary angiography should be performed on symptomatic patients and patients with severe coronary artery involvement. The angiography can accurately evaluate the coronary artery. Arterial stenosis and occlusion degree and distal lesions may cause serious complications such as transient myocardial ischemia and ventricular fibrillation due to blockage of the vascular lumen. The indications for coronary angiography are:

1 has myocardial ischemia symptoms,

2 persistent heart valve disease,

3X-line plain film shows coronary artery calcification,

4 echocardiography shows persistent coronary aneurysms.

2, gallbladder effusion occurs in the subacute phase, can occur severe abdominal pain, abdominal distension and jaundice, can be found in the right upper abdomen, abdominal ultrasound can confirm that most of the natural recovery, occasionally complicated by intestinal obstruction or intestinal bleeding .

3, arthritis or joint pain occurs in the acute or subacute phase, both large and small joints can be affected, about 20% of cases, with the condition improved and healed.

4, the acute phase of the nervous system changes include aseptic cerebrospinal meningitis, facial nerve paralysis, hearing loss, acute encephalopathy and febrile seizures, etc., caused by vasculitis, clinically common, rapid recovery, good prognosis, of which sterility Cerebrospinal meningitis is the most common, the incidence rate is about 25%, mostly occurs in the first 2 weeks of the disease, some children with increased intracranial pressure, the performance of the front bulge, a small number of children with stiff neck, may have lethargy, binocular gaze, coma and other consciousness Obstacles, cerebrospinal fluid lymphocytes increased slightly, sugar, chloride normal, protein accountants most normal, clinical symptoms disappeared within a few days, facial nerve paralysis more common in severe patients, often peripheral palsy, may be due to vasculitic reactions The facial nerve, or adjacent vascular lesions, such as aneurysm formation, arterial dilatation, etc., caused by transient compression of the facial nerve, due to limb spasm caused by stenosis or occlusion of the middle cerebral artery during recovery, it is easy to leave sequelae, relatively rare.

5, other complications of pulmonary vasculitis in the chest X-ray showed increased lung pattern or flaky shadow, occasional pulmonary infarction, acute stage may have urethritis, urinary sediment can be seen leukocytosis and mild proteinuria, iris eyelash Spondylitis is less common, about 2% of patients have a body aneurysm, with sputum, radial artery more common, occasional finger gangrene.

Kawasaki disease symptoms common symptoms sacral and dorsal edema lips cleft palate tachycardia nails visible transverse groove spinning persistent fever upper extremity edema systolic murmur characteristic toe end large piece of peeling maculopapular pulse acceleration

The main symptoms are common persistent fever, 5 to 11 days or longer (2 weeks to 1 month), body temperature often reaches above 39 °C, antibiotic treatment is ineffective, common bilateral conjunctival hyperemia, lip flushing, cleft palate or bleeding, see Yangmei-like Tongue, hard edema in the hand, flushing in the palm and sole of the foot early, 10 days after the characteristic toe-end large-scale peeling, appearing at the junction of the nail bed skin, as well as acute non-suppurative transient cervical lymph node swelling, the former neck The most prominent part is about 1.5cm in diameter, mostly appearing on one side, slightly tender, occurring within 3 days after fever, self-healing after a few days, and soon after fever (about 1-4 days), rash or polymorphous erythema A skin rash, occasionally a herpes-like rash, is more common in the trunk, but no herpes and crusting, and subsides in about a week.

Other symptoms include heart damage, symptoms of myocarditis, pericarditis and endocarditis, acceleration of the patient's pulse, tachycardia during auscultation, galloping, low heart bluntness, systolic murmurs are more common, can occur Valvular insufficiency and heart failure, for echocardiography and coronary angiography, can be found in most patients with coronary aneurysm, pericardial effusion, left ventricular enlargement and mitral regurgitation, X-ray chest X-ray enlargement, even See joint pain or swelling, cough, runny, abdominal pain, mild jaundice or aseptic cerebrospinal meningitis, about 20% of cases in the acute phase of the perineum, perianal skin flushing and desquamation in 1 to 3 years The original part of the pre-vaccinated BCG was used to reproduce erythema or crusting, and the nail was visible in the recovery period.

The length of the disease is different. The first stage of the disease is acute fever. The general course of disease is 1 to 11 days. The main symptoms appear after fever, and severe myocarditis can occur. The second phase is the subacute phase, generally the disease course is 11~ 21 days, most of the body temperature decreased, the symptoms eased, membranous peeling occurred on the toe end, severe cases continued to heat, coronary aneurysm occurred, which could lead to myocardial infarction, aneurysm rupture, most patients entered the third period in the fourth week The recovery period is generally 21 to 60 days, and the clinical symptoms subsided. If there is no obvious coronary artery disease, it will gradually recover. Coronary aneurysm is still sustainable, and myocardial infarction or ischemic heart disease may occur. A few severe coronary lesions Aneurysm patients enter the chronic phase, can be prolonged for several years, left coronary artery stenosis, angina pectoris, cardiac insufficiency, ischemic heart disease, can be life-threatening due to myocardial infarction.

According to the summary of 217 death cases by the Japanese MCLS Research Committee in 1990, in terms of pathological morphology, the vasculitis of this disease can be divided into four phases:

Phase I: about 1 to 2 weeks, characterized by:

1 small arteries, venules and microvessels and inflammation around them;

2 inflammation of the medium and aorta and its surroundings;

3 lymphocytes and other white blood cells infiltration and local edema.

Phase II: about 2 to 4 weeks, characterized by:

1 inflammation of small blood vessels is reduced;

2 to become the main inflammation of the middle arteries, more common coronary aneurysms and thrombosis;

3 major arteries are rare vascular inflammation;

4 monocyte infiltration or necrotic changes.

Phase III: about 4 to 7 weeks, characterized by:

1 small blood vessels and microvascular inflammation subsided;

2 medium arteries develop granuloma.

Stage IV: about 7 weeks or more, most of the acute inflammation of the blood vessels disappeared, replaced by thrombosis of the middle arteries, obstruction, thickening of the intima and aneurysm and scar formation. for:

1 medium or large artery outside the organ, more invading the coronary artery, iliac artery, radial artery and neck, chest, other arteries of the abdomen;

2 internal organs of the organ, involving the heart, kidney, lung, gastrointestinal, skin, liver, spleen, gonads, salivary glands and brain and other body organs.

In addition to vasculitis, pathology also involves a variety of organs, especially interstitial myocarditis, pericarditis and endocarditis, which can affect the conduction system, often leading to death in stage I lesions, to II, IV In the period of ischemic heart disease, myocardial infarction can cause death, as well as aneurysm rupture and myocarditis is also an important cause of death in stage II and III.

The vascular pathology of MCLS is very similar to that of infantile nodular polyarteritis. In addition to coronary or pulmonary aneurysm and thrombosis, the intima of the aorta, ileum artery or pulmonary artery changes. The fluorescent antibody can be seen in the myocardium and spleen. There are immunoglobulin IgG deposits in the arterial wall of lymph nodes, vasculitis in cervical lymph nodes and skin, accompanied by small vessel fibrotic necrosis, high thymus atrophy, increased heart weight, ventricular hypertrophy, and mild hepatic steatosis in the liver. As well as lymph node congestion and follicular enlargement, but the glomerulus has no significant lesions.

The Japanese MCLS Research Committee (1984) proposed that the diagnostic criteria for this disease should be determined by satisfying at least five of the following six major clinical symptoms:

1 Unexplained fever for 5 days or more;

2 bilateral conjunctival hyperemia;

3 oral and pharyngeal mucosa diffuse hyperemia, lip redness and chapped, and is a bayberry tongue; 4 early onset of the foot and foot hard swelling and palmar redness, and recovery of the finger toe end of the film peeling; 5 trunk erythema multiforme, But no blistering and crusting; 6 non-suppurative swelling of the cervical lymph nodes, the diameter of which is 1.5cm or more, but if two-dimensional echocardiography or coronary angiography to detect coronary aneurysm or dilation, then the four main symptoms are positive Can be diagnosed.

Kawasaki disease check

In the acute phase, the total number of white blood cells and the percentage of granulocytes increased, and the left side of the nucleus was shifted. More than half of the patients showed mild anemia, and the erythrocyte sedimentation rate increased significantly. The first hour was up to 100 mm, and the serum protein swimming showed an increase in globulin, especially 2 globulin. Significantly increased, albumin decreased, IgG, IgA, IgA increased, platelets increased in the second week, blood was hypercoagulable, anti-streptolysin O titer was normal, rheumatoid factor and anti-nucleus were negative, C The reaction protein is increased, the serum complement is normal or slightly higher, the urinary sediment can be seen with leukocytosis and/or proteinuria, and the electrocardiogram can be seen in various changes. The ST segment and T wave abnormalities are more common, and the PR, QR interval prolongation, abnormality can also be displayed. Q wave and heart rhythm disorder, two-dimensional echocardiography for cardiac examination and long-term follow-up in a number of diseases can be found in a variety of cardiovascular lesions such as pericardial effusion, left ventricular enlargement, mitral regurgitation and coronary dilatation or aneurysm formation It is best to check once a week in the acute and subacute phases of the disease. It is the most reliable non-invasive method for monitoring coronary aneurysms. In cases of aseptic meningitis, Lymphocytes in cerebrospinal fluid can be as high as 50 ~ 70 / mm3, in some cases, serum bilirubin or alanine is slightly higher, bacterial culture and virus isolation are negative results.

Diagnosis and identification of Kawasaki disease

It should be differentiated from various rash infectious diseases, viral infections, acute lymphadenitis, rheumatoid diseases and other connective tissue diseases, viral myocarditis, rheumatoid carditis.

The difference between this disease and scarlet fever is:

1 rash begins on the third day after the onset;

2 The rash morphology is close to measles and polymorphic erythema;

3 The age of good hair is the period of infants and young children;

4 penicillin has no effect.

The difference between this disease and juvenile rheumatoid disease is:

1 The fever period is shorter and the rash is shorter;

2 hand and foot are swollen, showing frequent flushing;

3 types of rheumatoid factor were negative.

The difference from exudative polymorphic erythema is:

1 eye, lip, no purulent secretion and pseudomembrane formation;

2 rash does not include blisters and scars.

There are many similarities with the symptoms of typical nodular arteritis (Kussmaul-Maier type), but the incidence of MCLS is more, the course of disease is shorter, and the prognosis is better. The difference is as follows: 1 The latter shows vasculitis Significant fibrinoide necrosis, and MCLS is rare in this necrotizing change or only slight change; 2 typical nodular arteritis is rare in the pulmonary artery.

The difference with systemic lupus erythematosus is:

1 rash is not noticeable on the face;

2 the total number of white blood cells and platelets generally increase;

3 anti-nuclear antibodies are negative,

4 good age is more common in infants and boys.

The differences from infant-type nodular polyarthritis and rash virus infection are:

1 lip flushing, chapped, bleeding, showing bayberry tongue;

2 hand and foot hard swelling, often flushing and late appearance of the toe end membranous peeling;

3 eye conjunctival edema or secretions;

4 The total number of white blood cells and the percentage of granulocytes increased, with the left shift of the nucleus;

6 ESR and C-reactive protein were significantly increased.

The difference with acute lymphadenitis is:

1 neck lymph node enlargement and tenderness is mild, local skin and subcutaneous tissue without redness; 2 no suppurative lesions.

The difference with viral myocarditis is:

1 coronary artery lesions are prominent;

2 characteristic hand and foot changes;

3 high fever continues to retreat.

The difference with rheumatic carditis is:

1 coronary artery lesions are prominent;

2 no meaningful heart murmur;

3 The age of onset is mainly infants and young children.

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