Cqs Disease Hotspot Forum Time: December 29, 2012 Source: Original site Click: 709 times I want to comment (0) "Font: Big Small" 12th of Kawasaki Disease clinical questions
1. What is the disease of Cqs disease?
Cqs disease is the preferred report of Kawasaki in 1967, and named after his name of the disease, also known as cutaneous mucosal lymph node syndrome (MCLS), clinical manifestations: fever, rash, non-purulent lymph node enlargement of the neck, eye-binding membrane congestion, oral mucosa diffuse congestion, Yangmei tongue, palm plantar erythema, hand-foot hard edema and so on.
2. Epidemiological characteristics of Cqs disease?
There were three onset peaks in Japan, 1979 in 15519 cases, 1982 15519 cases, 1986 12847 cases, 1973 was first found in the Korean peninsula outside Japan, China and the United States, Australia and Europe; June-November for the onset of peak, male morbidity is higher than female (1.35~1.5:1), recurrence rate of about 1~3%, Four seasons have onset, our country to spring and summer in a little more.
3. What are the manifestations of the main symptoms of Cqs disease?
The main symptoms of the performance rate: Fever lasts more than 5 days (a few cases within 5 days of fever-light disease), eye bulbar conjunctival congestion, mouth flushing, oral mucosa diffuse congestion, Yangmei tongue, multi-shape rash is often a project (performance rate >90%), non-purulent cervical lymph node enlargement (performance rate 50%~75%), Erythema (90%), hard edema (75%), and the skin transition of the finger-end nail-bed have small peeling (recovery period) for the required item (98%).
4. What diseases should be identified with Cqs disease?
⑴ Specific inflammatory disease (infectious): Yersinia pestis infection, hemolytic streptococcal infection (scarlet fever, fulminant), staphylococcus infection (toxic shock syndrome), Chlamydia pneumoniae infection, viral infection (measles, influenza, EBV, HIV), Candida albicans infection, leptospirosis.
⑵ non-specific inflammatory diseases: Stills disease, Stevons-johnson syndrome, drug allergy, after vaccination, after burns.
5. What are the main treatment methods for Cqs disease?
The main treatment is the acute stage of Kawasaki disease treatment, clear diagnosis after oral enteric-dissolving aspirin 30~50mg/kg.d, divided into two or three oral, hot back adjusted to 3~5mg/(kg D) and the course of the disease within 10 days (multi-claim 7 days) large doses of immunoglobulin static drops, at present, many international claims 2g/ KG single use.
6. What kind of patients need to use globulin therapy?
The American Heart Association (AHA), Cqs disease in the acute phase of children are used IVGG, Kawasaki Disease Research Group IVGG therapy indications for coronary artery tumor high-risk patients, more use Harada scoring method: ① white blood cell count >12x109/l;② platelet count <350x109/L; ③c-reactive Protein strong positive (>4.0MG/DL); ④ hematocrit <0.35;⑤ plasma albumin <35g/l; ⑥ age ≤ 12 months; ⑦ sex, male. The above scoring method scored within 7 days of onset, each of 1 points, divided into 4 points for ivgg therapy indications.
7. Intravenous immunoglobulin, the mechanism of the action?
⑴ closed the IGFC receptor on the surface of mononuclear cells, platelets or vascular endothelial cells in the blood, blocking the immune response of IGFC and IGFC receptors;
⑵ can make the anti-unique antibody repair;
⑶ provides a specific antibody, neutralizing antigen (toxin) effect;
⑷ inhibits PDGF (platelet-derived growth factor).
8. What is the mechanism of the traditional preferred therapeutic drug aspirin?
⑴ inhibits the production of prostaglandin by inhibiting also oxidase;
⑵ can block the production of thrombus A2 in platelets;
9. Can patients with Cqs disease use hormonal drugs?
For patients diagnosed with Kawasaki disease, the use of the hormone is limited to patients who continue to have fever after 2 or more intravenous globulin.
10. What is the natural course of Cqs disease?
The course of Cqs disease can be divided into the following four periods:
① Acute period 1-11 days;
② subacute phase: 11-21 days;
③ recovery period: 21-60 days;
④ Chronic Period: 60 days later.
11. What are the clinical manifestations of CQS disease in various stages?
Acute phase: high fever, eye-binding membrane congestion, rash, oral changes, swollen lymph nodes, irritability, erythrocyte sedimentation rate, CRP increase, white blood cells;
Subacute phase: Body temperature can still fluctuate, continue irritability, less than before, eye-binding membrane congestion is not cured, finger toe-end peeling, platelet increase;
Recovery period: The symptoms completely disappeared, the eye binding membrane slightly left congestion, lymph node swelling, ESR, white blood cell total gradually normal;
Chronic period: Most of the clinical manifestations are normal.
12. What are the characteristics of coronary artery and other arteries during the course of Cqs disease?
Acute phase: Peripheral inflammation of the arteries, microvascular and small arteries, small phlebitis, medium-sized and large-scale intima-inflammatory disease;
Subacute stage: Aneurysm, thrombosis, stenosis of medium artery, edema of blood vessel wall;
Recovery period: The inflammation of the blood vessels is relieved, the aneurysm can gradually subside;
Chronic period: scar formation, intima thickening.
13. What are the fatal causes of Cqs disease in various stages?
Acute stage: myocarditis, arrhythmia;
Subacute stage: Myocardial infarction, aneurysm rupture, myocarditis, arrhythmia;
Recovery period: myocardial infarction, ischemic heart disease;
Chronic stage: myocardial infarction.
14. Cardiovascular complications of Cqs disease?
The involvement of the heart and coronary arteries occurs 1-6 weeks, the cardiovascular complication mainly involves the middle and small arteries and the heart, especially the coronary artery is violated, some children can form coronary artery tumor, severe can cause myocardial infarction or even sudden death.
15. Criteria for coronary artery abnormalities in children:
The inner diameter of the coronary artery of 5 years old is >3mm,5 >4mm, the coronary artery diameter is 1.5 times times of the adjacent segment and the lumen is irregular.
Classification of coronary artery lesions: small coronary tumors or coronary dilatation: the inner diameter of the coronary artery of ≤4mm;≥5-aged children is less than 1.5 times times that of normal coronary artery dilatation; medium-sized coronary artery aneurysm: coronary diameter >4mm≤8mm , ≥ 5-year-old elder child coronary artery dilatation is normal 1.5~4 times; giant coronary aneurysm: coronary artery diameter >8mm; The internal diameter of coronary artery dilatation of ≥5-aged elder children is 4 times times larger than normal.
16. The incidence of coronary artery abnormalities in Cqs disease?
In untreated Kawasaki disease cases, the incidence of coronary dilatation is 18.6%~26.0%, more can be recovered in the course of 第3-5 weeks, the incidence of coronary artery tumors is 3.1%~5.2%, about half of the cases in the course of 1-2 years to subside, a small number of tumor body rupture. Because the local wall of the coronary artery has been fibrosis, intimal hyperplasia, it can not be effectively expanded, serious patients with thrombosis, stenosis of the lumen and even myocardial infarction, 5%~10% coronary lesions can develop into ischemic heart disease.
17. What is the degree of coronary lesion in Cqs disease?
| Grade |
Grading criteria |
| Ⅰ |
No expansion of the coronary arteries at any time |
| Ⅱ |
Temporary expansion in the acute period: only mild, temporary expansion of the coronary arteries and return to normal within 6-8 weeks of the course of the disease |
| Ⅲ |
1 small to medium size coronary artery tumors with single coronary artery |
| Ⅳ |
≥ 1 large coronary tumors (including giant coronary tumors), or multiple or complex aneurysms in a coronary artery but without obstruction |
| Ⅴ |
Coronary angiography shows stenosis or obstruction |
| Ⅴa |
No myocardial ischemia |
| Ⅴb |
Have myocardial ischemia |
18. Management of coronary artery lesions in Cqs disease?
| Hazard level |
Drug therapy |
Follow-up programme |
| Ⅰ, Ⅱ class |
The course of the disease 3 months discontinued aspirin |
Clinical follow-up 5 years, time for the course 1m,2m,6m,1y,5y follow-up ECG, echocardiography necessary chest film, the last follow-up recommended to do exercise ECG |
| Ⅲ class |
A small dose of aspirin lasts at least until the aneurysm subsides, and an additional antiplatelet drug is needed for medium-sized aneurysms |
Annual cardiac follow-up echocardiography, ECG, concurrent cardiovascular risk assessment and guidance, and every 2 years of 10-year-old patients undergoing stress tests or myocardial perfusion imaging |
| Ⅳ class |
Giant aneurysms require long-term aspirin (daily 3~5mg/kg) and warfarin (target inr:1.2~1.5) or low molecular weight heparin (target anti-factor ⅹa0.5~1.0u/ml) combined |
Every 6 months follow-up echocardiography, ECG, annual load test or myocardial perfusion imaging examination, recommended reproductive guidance for women of childbearing age |
| Ⅴa class |
The combination of small dosage aspirin and warfarin or low molecular weight heparin can be used to prevent ischemic attacks and cardiac insufficiency, and to apply beta-blockers, calcium channel blockers, ACEI, etc. |
Every 6 months cardiac follow-up echocardiography, ECG, annual load test or myocardial perfusion imaging examination, for women of childbearing age, recommended reproductive guidance |
| Ⅴb class |
Drug treatment at the same ⅴa level, according to the treatment of indications of the choice of bypass grafting or interventional treatment measures |
Lifelong follow-up, every 3-6 months/times, according to the condition at different follow-up time to choose a variety of different examinations for women of childbearing age, recommended reproductive guidance |
19. Cqs Post-illness vaccination management?
There are two important problems in the prevention and vaccination of children with Cqs disease, one is that the immunoglobulin block live viral vaccine replication and the acquired acquired immunodeficiency establishment; second, the safety of children who have been vaccinated against live or other vaccines in the recovery period of Cqs disease. Some autoimmune diseases, including systemic vasculitis, may suddenly worsen after the application of live or dead vaccines, so all vaccinations after Kawasaki disease are delayed for at least 3 months (generally recommended after June). The AHA recommends that non-intestinal live virus vaccination (measles, mumps and rubella) should be delayed for at least 5 months after the immunoglobulin (generally recommended after June), since passive antibodies may interfere with immune function. However, during the outbreak of measles, the measles vaccine should be used sparingly for children who had not previously had passive immunization, and the vaccine should be multiple-cropping later.
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