Kawasaki disease was first reported by Japanese Fukuzawa Yukichi in 65438-0967 and was named after him. Also known as cutaneous mucosal lymph node syndrome (MCLS), its clinical manifestations are fever, rash, neck non-suppurative lymphadenopathy, conjunctival congestion, diffuse congestion of oral mucosa, erythema of bayberry tongue, palm and sole, and hand and foot edema.
Scientific name of western medicine: Kawasaki disease
Kawasaki disease
Other names: cutaneous mucosal lymph node syndrome
Department: Internal Medicine-
Main symptoms: fever, rash, cervical non-suppurative lymphadenopathy, conjunctival congestion, diffuse congestion of oral mucosa, bayberry tongue.
Main reason: the reason is unknown.
Multiple groups: infants and young children
catalogue
Brief introduction of disease
cause of a disease
clinical picture
Diagnosis and differentiation
Disease treatment
Disease prognosis
expert opinion/advice
launch
Brief introduction of disease
Kawasaki disease was first reported by Japanese Fukuzawa Yukichi in 65438-0967 and was named after him. Also known as cutaneous mucosal lymph node syndrome (MCLS), its clinical manifestations are fever, rash, neck non-suppurative lymphadenopathy, conjunctival congestion, diffuse congestion of oral mucosa, erythema of bayberry tongue, palm and sole, and hand and foot edema.
There are three peaks in Japan, 1979 155 19 cases, 1982 155 19 cases,19861288 cases. Asians have more diseases, especially Japanese; Most of them occur in infants, with the peak in June-1 1. The incidence of male is higher than that of female (1.35-1.5:1), and the recurrence rate is about 1-3%, which occurs in all seasons. In China, the turn of spring and summer is slightly more. [ 1]
cause of a disease
The cause of Kawasaki disease is still unknown. According to the past few small epidemics, there have been cases of family illness, and many clinical manifestations are similar to acute infection, suggesting that there seems to be pathogens. However, due to the large number of boys, the incidence rate in Japan is high, and no direct pathogenic pathogen has been found so far, so the infection theory cannot be fully established. Streptococcus is the most concerned of all pathogenic bacteria, but streptococcus has never been isolated from children so far. It has also been mentioned that a pseudotuberculosis strain in Yersinia causes animal diseases? It seems to be related to Kawasaki disease, but there is no definite evidence. [2]
clinical picture
Disease symptoms
Clinically, high fever (above 39℃) is often the first manifestation, and the course of fever lasts for more than 5 days, usually for one or two weeks. Some fever goes down in a day or two, and the course of fever lasts for three or four weeks, and the antipyretics only drop slightly for a short time. After a few days of fever, the surface of palms and soles is red, swollen and painful, and maculopapules of different sizes appear on the trunk, with no special shape, but also on the face and limbs, without itching, herpes or scab. After a few days of fever, both conjunctivas were congested, especially bulbar conjunctiva, and only a few cases were complicated with suppurative conjunctivitis. Slit lamp can detect anterior iridocyclitis. Red, swollen, dry, chapped and even bleeding lips; The tongue is often bayberry tongue, and the oral mucosa is congested, but there is no ulcer. With fever, eyes, lips and rash, Kawasaki disease can basically be diagnosed.
In addition, 50% ~ 70% of Kawasaki disease has swollen lymph nodes in the early stage, and one or both sides are not suppurative, which subsides after a few days, and sometimes the swelling spreads to the submaxillary and is even misdiagnosed as parotitis. Lymphadenitis is confined to the anterior cervical triangle, painless and rarely spreads to other parts. In the second week of the course, 80% of the hands and feet peeled off. In order to start from the transition of nail bed, some babies can have perianal desquamation first, which needs special attention.
Disease staging
The course of Kawasaki disease can be divided into the following four stages:
① Acute phase 1- 1 1 day;
② Subacute stage: 1 1-2 1 day;
③ Recovery period: 265,438+0-60 days;
④ Chronic phase: 60 days later.
Staging symptom
Acute stage: high fever, conjunctival congestion, rash, oral changes, swollen lymph nodes, irritability, rapid ESR, high CRP and high white blood cells;
Sub-acute stage: the body temperature can still fluctuate, and it continues to be restless, which is lower than before. Conjunctiva hyperemia, toe desquamation and thrombocytosis.
Recovery period: symptoms disappeared completely, conjunctiva was slightly congested, lymph nodes were enlarged, erythrocyte sedimentation rate and total white blood cells gradually became normal;
Chronic phase: Most clinical manifestations are normal. [ 1][3]
Diagnosis and differentiation
disease diagnosis
The laboratory diagnosis of Kawasaki disease mainly depends on serological tests such as leukocytosis, platelet elevation, C-reactive protein elevation and accelerated erythrocyte sedimentation rate. At the same time, we should pay attention to whether it is accompanied by liver and kidney function damage and DD- dimer increase. In the auxiliary examination, special attention should be paid to the manifestations of echocardiography and electrocardiogram to indicate whether there are cardiovascular complications such as coronary artery dilatation and myocardial damage.
Diagnosis can refer to the following points:
1. If you have a fever for more than five days, you can make a diagnosis within five days if you have other signs, plus:
2. There are the following four articles:
(1) Bilateral conjunctiva is congested with no exudate.
(2) congestion of oropharynx mucosa, chapped lips and bayberry tongue.
(3) The hands and feet are red and swollen in the acute stage, and the skin around the nails desquamate in the subacute stage.
(4) The rash is mainly in trunk, maculopapular rash, erythema multiforme or bright red.
(5) The cervical lymph nodes are enlarged, and the diameter is more than 65438±0.5cm.
3. No other diseases can explain the above manifestations.
If fever is only accompanied by the other three, it can also be diagnosed as coronary artery aneurysm.
differential diagnosis
(1) Specific inflammatory diseases (infectious): Yersinia infection, hemolytic streptococcus infection (scarlet fever, fulminant), staphylococcal infection (toxic shock syndrome), Chlamydia pneumoniae infection, viral infection (measles, influenza, EBV, HIV), Candida albicans infection and leptospirosis.
⑵ Nonspecific inflammatory diseases: Stills disease, Stevons-Johnson syndrome, drug allergy, after vaccination, and after burn. [ 1][2]
Disease treatment
The main treatment of Kawasaki disease is oral enteric aspirin 30-50mg/Kg. On the second day after acute diagnosis, take it orally for 2-3 times and adjust it to 3-5mg/Kg. The day after the fever went down. During the course of 10 day (mostly within 7 days), large doses of gamma globulin should be injected intravenously in time. At present, a single dose of 2g/kg is advocated internationally. According to the plan of American Heart Association (AHA), it is suggested that children with Kawasaki disease should be given intravenous gamma globulin. However, Kawasaki disease research group in Japan thinks that the indication of intravenous immunoglobulin is high-risk patients with coronary artery aneurysm, and Harada score method is commonly used:
① White blood cell count >; 12× 109;
② Platelet count
③C-reactive protein was strongly positive (> 4.0 mg/dl);
④ Hematocrit
⑤ Plasma albumin
⑥ Age ≤ 12 months;
⑦ Sex, male.
The above scoring methods are all scored within 7 days of onset, each item is 1, and a score of more than 4 points is the indication of intravenous infusion of gamma globulin. [ 1]
Disease prognosis
The prognosis of Kawasaki disease is mostly good, and most children can recover by themselves. The symptoms are similar to those of the common cold, which is the main reason why Kawasaki disease is easily overlooked in the early stage. A few of them can produce complications, especially cardiovascular complications: coronary artery dilatation, serious aneurysm delayed for several years.
vaccination
There are two important problems in the vaccination of children with Kawasaki disease:
First, gamma globulin blocked the replication of live virus vaccine and the establishment of acquired immunity;
Secondly, the safety of children with Kawasaki disease after vaccination with live vaccine or other vaccines. Some autoimmune diseases, including systemic vasculitis, may suddenly worsen after the application of live or dead vaccines, so all vaccination after Kawasaki disease is postponed for at least 3 months (generally recommended after 6 months). AHA suggests that parenteral live virus vaccines (measles, mumps, rubella) should be delayed for at least 5 months after gamma globulin injection (generally after June), because passive immune antibodies may interfere with the immune function of immune agents. However, during the outbreak of measles, children who have not been passively immunized before should be cautiously applied measles vaccine in the early stage and vaccinated again in the future. [2][3]
expert opinion/advice
The diagnosis of typical cases of Kawasaki disease is clear, and the determination of atypical children is very important. Neglect is easy to miss diagnosis, and copying mechanically is easy to be misdiagnosed. Therefore, this situation needs to be carefully considered in combination with other laboratory tests, such as serum BNP and urine LDH. The most important thing is to pay strict attention to the changes of coronary artery in echocardiography.
Parents of children with Kawasaki disease should pay attention to the follow-up of children with Kawasaki disease, keep everything normal in time, and keep an annual health check-up, even after their children become adults. At present, many foreign reports believe that the occurrence of coronary heart disease in young adults is related to the childhood history of Kawasaki disease.
reference data
1
Du Zhongdong, Liang Lu, Meng Xiaoping, Li Tang, Zhang Tuohong, Kawasaki Zuo Fu, Liu Chuanyang, Beijing Children's Kawasaki Disease Epidemiological Investigation Collaboration Group. Epidemiological investigation of Kawasaki disease in hospitalized children in Beijing 1995- 1999. Chinese medical journal, 2003, 83 volumes, 2 1 issue.
2
, Qin,. Research progress on etiology of Kawasaki disease. International Journal of Pediatrics, Volume 34, No.03, 2007.
three
Shen Jie Li Jinkang Huang Min Yang Xiaodong Xu Meng Zhang Yongwei Liu Jiaying. Significance of changes of vascular endothelial growth factor in children with Kawasaki disease. China General Medicine, 20 10, Vol.8, No.06.
Open classification: