Skin mucous membrane lymph node syndromic ' Rugged disease in Sichuan)

Skin mucous membrane lymph node syndromic ' Rugged disease in Sichuan)

Summary


Skin mucous membrane lymph node syndromic ' Rugged disease in Sichuan) It is the bright whole body vascular inflammation syndrome of the reason, the artery while mainly influencing. Called baby's nodular many arteritis before 1967, Sichuan of Japan was rugged (Tomisaku Kawasaki) in 1967 The report, the whole world has illness coming on, in order to meet more with Asian descendant more children. The first rugged disease report of Sichuan of our country of 1976; The morbidity increases year by year, present and distribute or prevail small; Under the age of 5 to account for 80%, the mean age is 1.5 years old; Man: Female =1.5:1,The coronary artery happen and damage in 20%, then sending heart disease ranks first.


Cause of disease

The cause of disease is unclear

Infect: Bacterium, virus, Nick's body once,etc..

Hereditary background: Family have illness coming on and twins have illness coming on.


Pathology

Vascular inflammation, involve the small, middle, main artery, it is apt to involve the coronary artery.

I stage: 1~2 weeks, big, middle, inflammation around small vascular inflammation and blood vessel, the leucocyte soaks and edema; Take T lymphocyte as the core.

2One: 2~4 week, artery, elasticity fiber and the intersection of skin and layer rupture and necrosis, thrombosis influence mainly, aneurysm takes place.

One: 4~7 week, the intersection of China and artery take place granulation to be swollen, coronary artery part or totally blocking.

One: From 7 weeks to several years, the membrane is increased thick in the blood vessel, cicatrix appears, the obstructive artery can be openner.


The model behaves

Generate heat: Lasts 1~2 weeks or longer, antibiotic fails to respond to any medical treatment; It is congested that the ball combines the membrane, there is no suppuration secretion; Lip and oral cavity mucous membrane are congested, strawberry tongue, the lip chaps; Sole of the foot erythema of palm, the limb carries rigid edema, mean in convalescence the membrane of end of toe peels, the heavy one means the toenail can be lossed; Many shape rashes, filling the air erythema, anus week erythema and peeling; Enlargement of lymph nodes of neck.

The heart behaves: Pericarditis, myocarditis, membrane inflammation, rhythm of the heart not normal in the heart, coronary artery damage(the intersection of coronary artery and narrow or coronary artery and tumour) ,Myocardium infarction of a few.

Other manifestation: Among quality pneumonia, meningitis it is aseptic, digestive tract symptom, arthritis,etc..


Check in the laboratory

There is no specific laboratory inspection.

Hemology is checked: WBC ,Neutral cells ,The core moves to left; Slight anaemia; Blood platelet in 2~3 weeks ; The acute inflammatory reaction index rises.

Immunology is checked: Serum IgG ,IgA ,IgM ,IgE .

Electrocardiogram: ST-T changes.

Chest slice: Can present a quality change.

The interesting Fig. of the supersound: Acute one: It is obvious the pericardium accumulates the liquid, the left room is expanded, two, aorta one or three are returned to and flowed.

Coronary artery pathological change: Form of precededding arteriectasis, form of precededding aneurysm and coronary artery narrow.

The radiography of coronary artery: Check to reveal it is ischemic that coronary artery and tumour or ECG have cardiac muscle can choose use while displaying in supersound.


Diagnostic standard

Generate heat for more than 5 days, 4 items that have in the following 5 items can be made a definite diagnosis of

Four limbs: Sole of the foot erythema of palm, the limb is hard and swollen in end, mean the end of toe peels; Skin: Much shape erythema; The conjunctiva of eyes: Congested; Lip and oral cavity: The mucous membrane is congested, red bayberry something shaped like a tongue that the lip chaps; Cervical lymph-node: Swelling.

Fewer than 4 items, and there are persons who damages coronary artery that can also be made a definite diagnosis of.


Differential diagnosis

Septicaemia, exudative proteiform erythema, childhood rheumatoid arthritis ' Whole body type) , scarlet fever, suppurative lymphnoditis.


Treat

Control vascular inflammation:

Heavy dose of gamma globulin is intravenous to instil 1- 2g/kg, 8- 12hr note is finished, the onset is used in 10 days.

Enteric to dissolve aspirin 30- 50mg/( kgd) ,Heat steps back for 3 days and reduces to 3 gradually- 5mg/( kgd) ,It lasts 6- 8 weeks. The person who damages coronary artery should be lengthened, treated.

Glucocorticoid: Should not use alone, can consider adding and using the invalid one of gamma globulin.

Resist the blood platelet and assemble: Mi does not rattle away ' Pan ShengDing) 3- 5mg/( kgd) .

Treatment and surgery treatment to the ill: Liquid, protect liver, protect heart and support, treat, do the intersection of the coronary artery and skill of putting up a bridge if necessary.


Prognosis

For restricting by oneself disease, most prognosis is good, the aneurysm incidence of the form is 15%- 25% that the persons who do not undergo to treat effectively preceded, the majority precededs form aneurysm disappears in 1- 2 years, but can leave the tube wall increase thick and the function is unusual. The recuring rate is 1%- 2%. There is no person who precededs form aneurysm: Leave hospital, 1-3-6-2, act as in an all-round way checking 24 month ' Physical examination, EKG, UKG) ,Observe the trends closely when pathological change of persons who precededs form aneurysm is obvious, follow up a case by regular visits to, answer for 6- 12 months and follow up a case by regular visits to for a long time once in convalescence.


Skin Mucous membrane Lymph node Syndrome Rugged disease in Sichuan
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