BAYLEE LITTRELL: Info About Kawasaki Syndrome

Sunday, January 04, 2009

BAYLEE LITTRELL: Info About Kawasaki Syndrome
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Category: Life




that is the disease of kawasaki?


As all know our dear Baylee has been diagnosed with this disease, but what is? and some interesting data here.
KAWASAKI DISEASE

Introduction
The disease of Kawasaki (EK) must its name to the Japanese paediatrician that described east process in 1967, the Dr. Tomisaku Kawasaki. He previously recognized in a group of Japanese children a disease nondescribed that combined fever, eruption in the skin, conjuntivitis, enantema (reddening of the throat and the oral mucosa), swelling of the hands and feet, and increase as large as the lymphatic ganglia of the neck, that called Ganglionary Mucocutáneo Syndrome. Years later one began to describe the appearance of cardiac complications in children who had had the EK, including the development of aneurisms (expansion) of the coronary arteries.

What is?
The disease of Kawasaki is an acute systemic vasculitis; it means that an inflammation of the wall of the blood vessels takes place (vasculitis), of acute type, in more than a location (systemic). The majority of the children with EK presents/displays the symptoms without undergoing no complication, although in a small percentage the inflammation of the wall of the glass is followed of an expansion (aneurism) of the same; this complication preferably affects the coronary arteries, that are those that irrigates the heart.

How it frequents is?
The EK is a rare disease, in spite of being the second cause of vasculitis in paediatrics after the purple of Schönlein-Henoch. It is an almost exclusive disease of small children, so that of each 100 children with disease 80 they are smaller of 5 years. He is something more frequent in men than in children; also, he is more frequent at the end of the winter and in spring, although cases throughout all the year take place. It can affect children of all the races although he is much more frequent in Japanese children.

Which is the cause of the disease?
The cause of the disease of Kawasaki is not known, although it suspects that it has an infectious origin. It is probable that a germ (a virus or a bacterium) triggers, in patients predisposed genetically, an abnormal answer of his defensive system (immune system) responsible for an inflammatory reaction that can injure the wall of the blood vessels.

It is a hereditary disease? It is possible to be come up? She is contagious?
The disease of Kawasaki is not hereditary, although it suspects that a certain genetic predisposition can exist. He is exceptional that more of a member of a same family presents/displays the disease, that neither is contagious nor can be prepared. Rarely, a boy can get to have the EK twice.

Which are the most habitual symptoms?
The disease begins with high fever, for which is not cause, of at least 5 days of evolution, that can be accompanied or to follow itself of a conjuntival injection (red eye) nonexudative (without legaña). Besides the fever he is frequent that the children have an intense irritability, unusual in other febrile processes.
The boy can present/display different types from cutaneous eruptions, including eruptions similar to those of the measles, the scarlet fever or the urticaria. The eruption mainly affects the zone of the diaper, although it can extend to the trunk and the extremities.
The manifestations of the EK concerning the mouth include the presence of red, shining lips and fisurados, reddening of the language (so significant that "aframbuesada language" is called), and reddening of the pharynx.
Las Palmas and the plants can be intensely red, being able to also swell the hands and the feet. Between the second and third week it appears a moulting characteristic (in colgajos) around the end of the foot and fingers.
In more than half of the patients an increase of the size - of at least 1.5 cm of diameter takes place of some ganglion of the neck, habitually of only one.
Other symptoms that also can be present are pain or inflammation in the joints, abdominal pain, diarrhoea, irritability and headache, among others.
The affectation of the heart is the most serious manifestation of the EK by the possibility of producing complications in the long term. Cardiac blowings, echocardiographic arrhythmias and alterations can be detected. Inflammation in anyone of the components of the heart can exist, including the membrane that surrounds to the heart (pericarditis), the own cardiac muscle (miocarditis), or the cardiac valves (endocarditis). The most important characteristic of this disease, nevertheless, is the appearance of aneurisms in the coronary arteries.

It is the equal disease in all the children?
No. Neither the gravity of the disease is the same, nor all the described manifestations are present in each one of the children with EK. In fact, the cardiac complications are infrequent, since only 2 of each 100 children suitably treated have aneurisms.
Also incomplete forms of the disease have communicated, mainly in smaller children of 1 year; it means that the children do not have characteristic the clinical manifestations, which does more difficult to arrive at the correct diagnosis. This group of children is the one that, in addition, has more risk of developing aneurisms.

It is the equal disease in children and adults?
The EK is a disease of the childhood. The adults can present/display similar forms of vasculitis, but with different a clinical picture.

How diagnostic?
The definitive diagnosis is always based on clinical criteria. For it the presence of high fever - for which is not cause of 5 or more days of duration, and 4 of the following 5 manifestations are demanded: bilateral conjuntivitis, cutaneous eruption, alterations in the mouth, alterations in the extremities, and increase of size of the lymphatic ganglia. In order to be able to make the diagnosis it is precise that there is not evidence of no other disease that can produce similar symptoms.
Given the existence of incomplete forms of the disease always the diagnosis of EK in those children with suggestive symptoms of the same must be considered, although they do not fulfil the criteria mentioned diagnoses.

What analysis or what tests are useful?
The analyses available are not specific of the disease but they turn out useful to evaluate the degree of existing inflammation. Some of the used indicators of inflammation include the elevation of the sedimentation velocity (generally more discharge than in other febrile diseases of characteristic similars), of the number of white blood cells of the blood (leucocytosis), and the diminution of red blood cells (anemia). The number of plaquetas (cells in charge of the coagulation of the blood) is normal in the first week of the disease, but it increases as of the second week reaching very high numbers. He is recommendable to repeat the analyses periodically until they are standardized.
Also it agrees to ask for an electrocardiogram (ECG) and ecocardiograma. Ecocardiograma allows to detect the presence of aneurisms, since the form evaluates so much as the size of the coronary arteries. When a boy presents/displays coronary alterations requires pursuit by infantile cardiology, as well as additional studies and evaluations.

He has cures?
The majority of the children with disease of Kawasaki is cured. Nevertheless, some children develop cardiac complications in spite of to be treated suitably. The EK cannot be prepared, reason why the best way to diminish their cardiac complications is the precocious diagnosis and treatment.

Which is its treatment?
Once one becomes the diagnosis - probable or definitive from EK one is due to come to the hospitable entrance from the boy for the treatment and valuation from the possible cardiac complications. He is recommendable that the children are treated as soon as the diagnosis is realised, since it avoids or reduces the development of complications.
The treatment consists of the administration of aspirina via oral and intravenous gammaglobulina, both to high doses. The gammaglobulina contains a concentrate of the present human antibodies in the blood. This treatment will diminish the systemic inflammation, making disappear the acute symptoms. The gammaglobulina forms the base of the treatment, since he is able to prevent the appearance with coronary alterations in a great proportion of patients. Also they are used, although with less frequency, the corticoids.

Which are the main indirect effect of the treatment?
Aspirina can cause gastric intolerance, as well as a transitory elevation of hepatic enzymes. The gammaglobulina is generally very well tolerated.

How long must last the treatment?
The great majority of the patients will receive a single dose of gammaglobulina, although in some children it is necessary to repeat his administration.
Aspirina is administered to high doses during the first days of the EK, while the fever persists; as soon as this one disappears stoops the dose. Later it is continued administering aspirina to low doses by his effect like plaquetario antiagregante, that is to say, so that plaquetas others do not stick an a and the formation of trombos or clots within the aneurisms is avoided. The appearance of trombos can favor the development of a myocardium infarct that, although is something absolutely exceptional, represents the most frightful complication of the EK.
The duration of the treatment with aspirina depends on the presence or not on aneurisms. Those that does not develop them will use aspirina during few weeks, whereas those that have yes them use it during longer periods.

What paper has nonconventional the alternative treatments/?
These treatments are not applicable in this disease.

What type of revisions and periodic controls is necessary?
It is recommended periodically to obtain a haemogram (red blood cell counts, white and plaquetas) with sedimentation velocity, until they are standardized.
Also it is necessary to realise ecocardiogramas seriados to detect the presence of coronary aneurisms, as well as to follow his evolution. The frequency whereupon will have to be realised will as large as depend on the presence and the aneurisms. The majority of the aneurisms tends to improve and to disappear.
The people in charge of the pursuit of these children are the paediatrician, the cardiologist and the infantile rheumatologist. In some places where there is no infantile rheumatologist, the paediatrician along with the cardiologist will have to become position of the pursuit of these patients, of whom they especially have cardiac affectation.

How long lasts the disease?
The disease of Kawasaki consists of three stages:
1) Acute. It includes the first 2 weeks of disease, when the clinical fever and the other manifestations are present;
2) Subaguda. It includes/understands of the second the fourth weeks, and is the period in which the number of plaquetas increases and can appear the aneurisms;
3) Convalecencia. One extends between the first one and third month, when all the analyses return to normal values and the alterations of the blood vessels (aneurisms of the coronary arteries) disappear or fall of size.

Which is its prognosis?
The majority of the patients has an excellent prognosis and will have an absolutely normal life, growth and a development.
The prognosis of the patients with persistent coronary alterations will especially depend on the development of estenosis (reduction of the diameter of the blood vessel) or occlusions (obstruction) of the glasses by formation of clots or trombos.

How it affects the daily activities? It is possible to be vaccinated to him? It can practice sports?
It is recommended not to vaccinate to these children in the 6 months following to the EK since as much the own disease as, mainly, the gammaglobulina administration, affects the answer of the immunological system of the boy.
The children without cardiac complications do not have any type of physical restriction for the sport practice or to realise any other activity of the daily life. The children with coronary aneurisms, nevertheless, will have to consult to the infantile cardiologist about their participation in sport activities of competition, especially during the adolescence

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