兒科川崎病 課件

1、Kawasaki Disease mucocutaneous lymph node syndromeLi FuhaiPediatric department of Qilu Hospital, Shandong UniversityFirst described by Dr. Tomisaku Kawasaki in Japan in 1967EtiologyRemains unknownInfectious origin, Genetic role,KD-associated antigenSuperantigen (toxic shock toxin, exfoliative toxin,

2、 streptococcal pyrogenic exotoxins) the role of regulatory T cells, chemokines, and Toll-like receptors HSP 56 (heat shock protein) simulates Autoantigen (HSP63), -antigen resemblance Vigor-ous antibody response common environmental triggersEPIDEMIOLOGYAsian and Pacifc Islander children are at highe

3、r risk for KDKD is an illness of early childhood, as the median age of illness is 2-3 yr and 80% of children are 5 yr KD may occur in adolescents.highest risk for coronary artery bnormalities:age, male genderlaboratory abnormalities including neutrophilia, thrombocytopenia, hepatic transaminase elev

4、ation, hyponatremia, hypoalbuminemia, and elevated C-reactive protein levels. Asian and Pacifc Islander race and Hispanic ethnicity Prolonged feverPATHOLOGYsevere vasculitis of all blood vessels predominantly affecting the medium-sized arteries, with striking predilection for the coronary arteries.

5、Pathologic examination reveals edema of endothelial and smooth muscle cells with intense inflammatory infiltration of the vascular wall, initially by polymorphonuclear cells but rapidly changing to macrophages, lymphocytes (which are mostly CD8 T cells), and plasma cells. IgA plasma cells are promin

6、ent in the inflammatory infiltrate. inflammation involves all three layers of the vascular wall, with destruction of the internal elastic lamina. The vessel loses its structural integrity and weakens, resulting in dilatation or aneurysm formation. Thrombi may form in the lumen and obstruct blood flo

7、w. In the healing phase, the lesion becomes progressively fibrotic, with marked intimal proliferation, which may result in stenotic or occlusion.PathologyPathologyPhase 1 1-9 day from the onsetCharacteristics: acute inflammatory alteration around small arteries. Small nutrient artery and vein of the

8、 main coronary artery wall were involvedInflammatory was occurred in pericardium , mesenchymal of myocardium and endocardium, infiltration of neutrophil , eosinophil , lymphocytePathologyPhase 210-21 daysinflammation involves all three layers of the vascular wall of meddle size coronary artery, with

9、 destruction of the internal elastic lamina and smooth muscle layer. Thrombi and Aneurysm formedPathologyPhase 328-31 daysVasculitis regressed the lesion becomes progressively fibrotic, with marked intimal proliferation, which may result in stenotic occlusion of the vessel over timePathologyPhase 4L

10、asted yearsThe lesion healed, myocardium cicatrix formed, occluded artery recanalized Small coronary artery with segmental necrosis and inflammation in a patient with Kawasaki disease. Clinical ManifestationsmajorFever : generally high spiking (to 104F or higher), unremitting , and unresponsive to a

11、ntibiotics.The duration of fever is generally 12 wk without treatment but may persist for 34 wk. Prolonged fever has been shown to be a risk factor for the development of coronary artery disease.bilateral bulbar conjunctival injection with limbal sparing, usually without exudate.erythema of the oral

12、 and pharyngeal mucosa “strawberry” tongue and dry, cracked lips; erythema and edema of the hands and feet; rash of various forms (maculopapular, erythema multiforme, or scarlatiniform) with accentuation in the groin area; appears 2-4 days after onset of the disease, lasts 4-5 days. nonsuppurative c

13、ervical lymphadenopathy, usually unilateral, with a node size of 1.5 cm or greater in diameter. Perineal desquamation is common in the acute phase. Periungual desquamation of the fingers and toes begins 13 wk after the onset of illness and may progress to involve the entire hand and foot.Clinical Ma

14、nifestationsminorCardiovascular fndings:Congestive heart failure, myocarditis, pericarditis, valvular regurgitationCoronary artery abnormalitiesAneurysms of medium-sized noncoronary arteriesRaynaud phenomenonPeripheral gangreneCardiac involvementCardiac involvement is the most important manifestatio

15、n of Kawasaki disease. Myocarditis manifested by tachycardia and decreased ventricular function occurs in at least 50% of patients. Pericarditis with a small pericardial effusion is common during the acute illness. Cardiac involvementCoronary artery aneurysms generally develop in up to 25% of untrea

16、ted patients during the 2nd3rd wk of illness and can be detected by two-dimensional echocardiography. Valvular regurgitation and systemic artery aneurysms may occur but are uncommon. Giant coronary artery aneurysms (=8?mm internal diameter) pose the greatest risk for rupture, thrombosis or stenosis,

17、 and myocardial infarction.Musculoskeletal system:Arthritis, arthralgiasGastrointestinal tract:Diarrhea, vomiting, abdominal painHepatic dysfunctionHydrops of gallbladderCentral nervous system:Extreme irritabilityAseptic meningitisSensorineural hearing lossGenitourinary system:Urethritis/meatitisOth

18、er fndings:erythema and induration of BCG scarAnterior uveitis (mild)Desquamating rash in groinclinical phasesKawasaki disease is generally divided into three clinical phases.The acute febrile phaseThe acute febrile phase, which usually lasts 12 wk, is characterized by fever and the other acute sign

19、s of illness. The subacute phaseThe subacute phase begins when fever and other acute signs have abated, but irritability, anorexia, and conjunctival injection may persist. The subacute phase is associated with desquamation, thrombocytosis, the development of coronary aneurysms, and the highest risk

20、of sudden death. This phase generally lasts until about the 4th wk. The convalescent phaseThe convalescent phase begins when all clinical signs of illness have disappeared and continues until the erythrocyte sedimentation rate (ESR) returns to normal, approximately 68 wk after the onset of illness.L

21、aboratory examinationThe white blood cell count is normal to elevated, with a predominance of neutrophils and immature forms.Elevated ESR, C-reactive protein, and other acute phase reactants are almost universally present in the acute phase of illness and may persist for 46 wk. The platelet count is

22、 generally normal in the 1st wk of illness and rapidly increases by the 2nd3rd wk of illness, sometimes exceeding 1,000,000/mm3. Sterile pyuria, mild elevations of the hepatic transaminases, and cerebrospinal fluid pleocytosis may be present.IgG,IgM,IgE,IgA and circulating immune complex increased.L

23、aboratory examinationECG:Sinus tachycardia ,nonspecific ST-T alteration.Two-dimensional echocardiography : to monitor the potential development of coronary artery abnormalities performed at diagnosis and again after 23 wk of illness. If results of both of these are normal, a repeat study is performe

24、d 68 wk after onset of illness. If coronary abnormalities are not detected by echocardiography by 68 wk after onset of illness, when the ESR has normalized, additional follow-up studies are optional.Aneurysms have been defned with use of absolute dimensions by the Japanese Ministry of Health and are

25、 classifed as: small (8 mm internal diameter)For patients who develop coronary artery abnormalities, more frequent echocardiographic studies and potentially angiography may be indicated.Coronary artery angiography :when ultrasonic echocardiogram show aneurysm or ECG indicates myocardial ischemia ,an

26、giography is recommended .Diagnosis criteriaFever lasting for at least 5 days*Presence of at least four of the following five signs:1.Bilateral bulbar conjunctival injection, generally nonpurulent2.Changes in the mucosa of the oropharynx, including injected pharynx, injected and/or dry fissured lips

27、, strawberry tongue3.Changes of the peripheral extremities, such as edema and/or erythema of the hands or feet in the acute phase; or periungual desquamation in the subacute phase4.Rash, primarily truncal; polymorphous but nonvesicular5.Cervical adenopathy, =1.5?cm, usually unilateral lymphadenopath

28、yThe diagnosis of KD is based on the presence of characteristic clinical signs. For classic KD, the diagnostic criteria require the presence of fever for at least 4 days and at least four of fve of the other principal characteristics of the illness.In atypical or incomplete KD, patients have persist

29、ent fever but fewer than four of the fve characteristics. In these patients, laboratory and echocardiographic data can assist in the diagnosis Incomplete cases are most frequent in infants, who, unfortunately, also have the highest likelihood of development of coronary artery abnormalities.DIFFERENTIAL DIAGNOSISscarlet fev