Edited and compiled by Yimaitong. Please do not reprint without authorization. Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome, was reported and named by Tomisaku Kawasaki of Japan in 1967. The cause is unknown and it mostly affects children. KD is a self-l

Yimaitong. Please do not reprint without authorization.

Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome, was reported and named by Tomisaku Kawasaki of Japan in 1967. The cause is unknown and it mostly affects children. KD is a self-limiting disease, most of which have a good prognosis. If not treated promptly, about 25% of children may develop coronary artery aneurysms (CAA), which may lead to coronary artery stenosis, acute myocardial infarction, or even death. . Therefore, early identification, timely diagnosis and treatment are particularly important.

Classification of Kawasaki disease

According to the degree of clinical manifestations of KD, it can be divided into: complete KD (CKD), incomplete KD (IKD), atypical KD (AKD), and Kawasaki disease shock syndrome (KDSS).

Clinical features of Kawasaki disease

Main clinical features of KD

1. Fever;

2. Bilateral bulbar conjunctival congestion;

3. Changes in the lips and oral cavity: red lips, strawberry tongue, diffuse congestion of the oropharyngeal mucosa;

4. Rash ( Including redness at the site of BCG vaccination);

5. Changes in the extremities: redness and swelling of the hands and feet in the acute stage, and periungual peeling in the recovery stage;

6. Non-suppurative cervical lymphadenopathy.

Other significant clinical features

7. Elevated liver transaminases early in the course of the disease;

8. Leukocytosis in urinary sediment in infants ;

9. Thrombocytosis in the recovery period;

10. Elevated BNP or NT-proBNP;

11. Echocardiography Shows mitral regurgitation or pericardial effusion ;

12. Gallbladder enlargement (gallbladder effusion);

13. hypoalbuminemia or hyponatremia .

Diagnosis of Kawasaki disease

Diagnosis of CKD

➤ is consistent with 5-6 clinical features;

➤ is consistent with 4 clinical features, and echocardiography shows coronary artery abnormalities.

Diagnosis of IKD

➤ meets 3 or 4 clinical features, no coronary artery dilation is found, but has some of the "other meaningful clinical features", other diseases are excluded, and the diagnosis is IKD;

➤ meets 3 clinical features, Echocardiography shows coronary artery abnormalities, and other febrile diseases are excluded, and IKD is diagnosed;

➤ has only 1 or 2 major clinical features, and other diagnoses are excluded, and IKD can also be considered.

2017 American College of Cardiology IKD diagnostic flow chart

Note: ESR: erythrocyte sedimentation rate ; ALT: alanine aminotransferase; LAD: left anterior descending coronary artery; RCA: right coronary artery

Diagnosis of AKD

Atypical manifestations of AKD Mainly include:

1. Nervous system manifestations: the most common is facial nerve paralysis, followed by aseptic meningitis, sensorineural deafness , ischemic stroke , increased cerebrospinal fluid cell count, epilepsy Attacks , quadriplegia, irritability, etc.;

2. Renal manifestations: the most common sterile pyuria, others include hemolytic uremic syndrome, nephrotic syndrome , acute interstitial nephritis , etc.;

3 . Rheumatic immune system manifestations: including arthritis, joint swelling and pain, myositis, etc.;

4. Digestive system manifestations: the most common is gallbladder edema, others include intestinal obstruction, pancreatitis, jaundice, abdominal pain, Vomiting, diarrhea, etc.;

5. Pulmonary manifestations: bronchopneumonia, pleural effusion, pneumothorax, interstitial pneumonia, cough, runny nose, posterior pharyngeal wall edema, chest X-raypulmonary infiltration, etc. ;

6. Cardiovascular system: abnormal extra heart sounds , electrocardiogram changes, peripheral artery aneurysms other than coronary arteries (axillary artery, etc.);

7. Blood system: elevated erythrocyte sedimentation rate, anemia;

8. Skin changes: small pustular rash , Nail horizontal lines.

KDSS Diagnosis

KDSS diagnostic criteria: On the basis of KD, there are symptoms of hypotension or hypoperfusion, such as tachycardia , prolonged capillary refill time, cool limbs, weak pulse, decreased urine output or Obstruction of consciousness occurs.

KDSS characteristics:

1. A larger proportion of women;

2. The average age is greater than that of ordinary KDs;

3. A higher proportion of neutrophils;

4. Higher CRP;

5. Hypoplateletemia

6. Hypohemoglobinaemia

7. Cardiac systolic function Disorders;

8. Coronary artery dilation and high incidence of gamma globulin unresponsiveness.

References:

[1] Pan Silin, Liu Fang, Luo Gang. Interpretation of key points of Japan’s "6th revised edition of the Japanese Diagnostic Guidelines for Kawasaki Disease" [J]. Chinese Journal of Practical Pediatrics, 2020, 35(11):846- 849.DOI:10.19538/j.ek2020110606.

[2] Zhang Qingyou, Du Junbao. Several issues in the diagnosis of Kawasaki disease[J]. Chinese Journal of Practical Pediatrics, 2020, 35(13):961-964.

[3 ] Lin Yao, Li Xiaohui, Shi Lin, et al. Interpretation of the 2017 edition of "Diagnosis, Treatment and Long-term Management of Kawasaki Disease—American Heart Association's Scientific Statement for Medical Professionals" [J]. Chinese Journal of Practical Pediatrics, 2017, 32 (09):641-648.

[4]Li Xiaohui. New progress in diagnosis and treatment of Kawasaki disease[J]. Chinese Journal of Practical Pediatrics, 2013(01):9-13.