Understanding Kawasaki Disease
Kawasaki Disease is an acute vasculitis (disorder involving inflammation of one or more blood vessels) which typically affects young children. Most often, the patients are young boys or girls in the first three years of life. After the age of 10 years, Kawasaki Disease is extremely rare, and the diagnosis should be regarded with suspicion.
Symptoms
The disease usually begins with a fever, unresponsive to Tylenol or aspirin, which continues for at least 7 - 10 days. Many - but not all - children develop a swollen area on the neck which looks like an infection. Doctors usually recognize the disease when they see an unresponsive fever accompanied by a rash; dry, cracked lips or other changes of the mouth or tongue; inflamed eyes (conjunctivitis); and arthritis or heart changes without other explanation.
The most important part of Kawasaki disease is its tendency to cause inflammation of the coronary arteries, which supply blood to the heart. Changes can be found on echocardiogram in about 15% of patients. A few of these patients will have aneurysms (balloon-like dilatations) that can be very serious. If one of the aneurysms becomes blocked or bursts, the blood flow to the heart is interrupted and the child can have a heart attack. Fortunately, this is very rare, and deaths due to Kawasaki disease are less than 5/1000.
Treatment
Treatment for Kawasaki disease is intravenous (IV) gammaglobulin. Large doses of intravenous gammaglobulin are usually very effective in stopping the fever of Kawasaki disease and seem to limit or prevent aneurysm formation as well. It is very rare for a child not to improve with gammaglobulin. Although some children require two treatments, if there is no improvement, the diagnosis should be reconsidered.
Other diseases can be confused with Kawasaki disease, which may require different therapy. Some doctors are reluctant to give gammaglobulin if the symptoms have been present for more than 10 days. In truth, gammaglobulin is still effective after the first 10 days, but the likelihood of preventing aneurysms is lower than if it is given during the first ten days. Recent studies have shown that corticosteroids are effective for many of the children who fail to respond appropriately to gammaglobulin.
Once the acute inflammation is brought under control, the chronic symptoms of Kawasaki disease are usually controlled with nonsteroidal anti-inflammatory drugs like naproxen or ibuprofen. Many cardiologists and some rheumatologists still use aspirin, but the other nonsteroidal drugs are considered safer, easier to give, and equally effective.
Where does Kawasaki Disease come from?
The etiology (origin) of Kawasaki disease is unknown. There have been many theories, but there are none that are generally accepted. It is not contagious to other children, although there are a few cases of brothers and sisters developing the disease. In rare cases, a few children have had two episodes. The disease often occurs in epidemics that occur every few years. In the winter of 2002, for example, the author received an increased influx of inquiries about the disease.
Recovery
Usually, children recover completely with proper therapy. There is some concern that children with damaged coronary arteries will have problems later in life, but this seems to be very rare. If your child has Kawasaki disease, he will need to be followed by his primary doctor, a cardiologist, and perhaps a rheumatologist or other specialist (depending on the symptoms).
The vast majority of children with Kawasaki disease recover and resume normal lives. Your doctor should be able to tell you if there is any reason to be unusually concerned. If your child has not recovered appropriately after an episode of Kawasaki disease, it is important to be sure that the diagnosis is right and that another disease requiring different therapy has not been missed. A pediatric rheumatologist is the specialist best able to do this for you.
Showing posts with label KD Info. Show all posts
Showing posts with label KD Info. Show all posts
Saturday, May 2, 2009
What Is Kawasaki Disease!
What is Kawasaki Disease?
Kawasaki disease is an illness that involves the skin, mouth, and lymph nodes, and most often affects kids under age 5. The cause is unknown, but if the symptoms are recognized early, kids with Kawasaki disease can fully recover within a few days. Untreated, it can lead to serious complications that can affect the heart.
Kawasaki disease occurs in 19 out of every 100,000 kids in the United States. It is most common among children of Japanese and Korean descent, but can affect all ethnic groups.
Signs and Symptoms
Kawasaki disease can't be prevented, but usually has telltale symptoms and signs that appear in phases.
The first phase, which can last for up to 2 weeks, usually involves a persistent fever higher than 104° Fahrenheit (39° Celsius) and lasts for at least 5 days.
Other symptoms that typically develop include:
severe redness in the eyes
a rash on the stomach, chest, and genitals
red, dry, cracked lips
swollen tongue with a white coating and big red bumps
sore, irritated throat
swollen palms of the hands and soles of the feet with a purple-red color
swollen lymph nodes
During the second phase, which usually begins within 2 weeks of when the fever started, the skin on the hands and feet may begin to peel in large pieces. The child also may experience joint pain, diarrhea, vomiting, or abdominal pain. If your child shows any of these symptoms, call your doctor.
Complications
Doctors can manage the symptoms of Kawasaki disease if they catch it early. Symptoms often disappear within just 2 days of the start of treatment. If Kawasaki disease is treated within 10 days of the onset of symptoms, heart problems usually do not develop.
Cases that go untreated can lead to more serious complications, such as vasculitis, an inflammation of the blood vessels. This can be particularly dangerous because it can affect the coronary arteries, which supply blood to the heart.
In addition to the coronary arteries, the heart muscle, lining, valves, and the outer membrane that surrounds the heart can become inflamed. Arrhythmias (changes in the normal pattern of the heartbeat) or abnormal functioning of some heart valves also can occur.
Diagnosis
No one test can detect Kawasaki disease, so doctors usually diagnose it by evaluating the symptoms and ruling out other conditions.
Most kids diagnosed with Kawasaki disease will have a fever lasting 5 or more days and at least four of these symptoms:
redness in both eyes
changes around the lips, tongue, or mouth
changes in the fingers and toes, such as swelling, discoloration, or peeling
a rash in the trunk or genital area
a large swollen lymph node in the neck
red, swollen palms of hands and soles of feet
If Kawasaki disease is suspected, the doctor may order tests to monitor heart function (such as an echocardiogram) and might take blood and urine samples to rule out other conditions, such as scarlet fever, measles, Rocky Mountain spotted fever, juvenile rheumatoid arthritis, or an allergic drug reaction.
Treatment
Treatment should begin as soon as possible, ideally within 10 days of when the fever begins. Usually, a child is treated with intravenous doses of gamma globulin (purified antibodies), an ingredient of blood that helps the body fight infection. The child also might be given a high dose of aspirin to reduce the risk of heart problems.
Kawasaki disease is an illness that involves the skin, mouth, and lymph nodes, and most often affects kids under age 5. The cause is unknown, but if the symptoms are recognized early, kids with Kawasaki disease can fully recover within a few days. Untreated, it can lead to serious complications that can affect the heart.
Kawasaki disease occurs in 19 out of every 100,000 kids in the United States. It is most common among children of Japanese and Korean descent, but can affect all ethnic groups.
Signs and Symptoms
Kawasaki disease can't be prevented, but usually has telltale symptoms and signs that appear in phases.
The first phase, which can last for up to 2 weeks, usually involves a persistent fever higher than 104° Fahrenheit (39° Celsius) and lasts for at least 5 days.
Other symptoms that typically develop include:
severe redness in the eyes
a rash on the stomach, chest, and genitals
red, dry, cracked lips
swollen tongue with a white coating and big red bumps
sore, irritated throat
swollen palms of the hands and soles of the feet with a purple-red color
swollen lymph nodes
During the second phase, which usually begins within 2 weeks of when the fever started, the skin on the hands and feet may begin to peel in large pieces. The child also may experience joint pain, diarrhea, vomiting, or abdominal pain. If your child shows any of these symptoms, call your doctor.
Complications
Doctors can manage the symptoms of Kawasaki disease if they catch it early. Symptoms often disappear within just 2 days of the start of treatment. If Kawasaki disease is treated within 10 days of the onset of symptoms, heart problems usually do not develop.
Cases that go untreated can lead to more serious complications, such as vasculitis, an inflammation of the blood vessels. This can be particularly dangerous because it can affect the coronary arteries, which supply blood to the heart.
In addition to the coronary arteries, the heart muscle, lining, valves, and the outer membrane that surrounds the heart can become inflamed. Arrhythmias (changes in the normal pattern of the heartbeat) or abnormal functioning of some heart valves also can occur.
Diagnosis
No one test can detect Kawasaki disease, so doctors usually diagnose it by evaluating the symptoms and ruling out other conditions.
Most kids diagnosed with Kawasaki disease will have a fever lasting 5 or more days and at least four of these symptoms:
redness in both eyes
changes around the lips, tongue, or mouth
changes in the fingers and toes, such as swelling, discoloration, or peeling
a rash in the trunk or genital area
a large swollen lymph node in the neck
red, swollen palms of hands and soles of feet
If Kawasaki disease is suspected, the doctor may order tests to monitor heart function (such as an echocardiogram) and might take blood and urine samples to rule out other conditions, such as scarlet fever, measles, Rocky Mountain spotted fever, juvenile rheumatoid arthritis, or an allergic drug reaction.
Treatment
Treatment should begin as soon as possible, ideally within 10 days of when the fever begins. Usually, a child is treated with intravenous doses of gamma globulin (purified antibodies), an ingredient of blood that helps the body fight infection. The child also might be given a high dose of aspirin to reduce the risk of heart problems.
Aspirin and Kawasaki Disease
Aspirin: The Wonder Drug
Aspirin: The Wonder Drug
Aspirin (medically known as acetylsalicylic acid) is a common household medication for pain (analgesic), fever (antipyretic), and inflammation (anti-inflammatory). This "simple" and inexpensive drug is so much underrated and practically taken for granted. Aspirin is really a versatile drug, with a lot of uses, much more than the lay public realizes. Inn the United States alone about 20 tons of aspirin is used each year.
How did aspirin come about?
In 1827, Leroux of France first discovered salicin, an active ingredient in the willow bark, and in 1838, Piria produced salicylic acid from salicin. In 1899, Dreser introduced acetylsalicylic acid (aspirin) into medicine. This has been in popular use since then.
Why is aspirin a Wonder Drug?
Aspirin was a labeled a Miracle Drug or a Wonder Drug when it was first introduced more than 100 years ago, and continues to deserve such a prestigious position in the physician's armamentarium because of its versatility. Other Miracle or Wonder Drugs when they were first introduced include Penicillin, Steroids, Chemo-therapeutic Agents for cancers, and lately, Viagra, for male erectile dysfunction.
How is aspirin versatile?
Besides being used as an analgesic for pain of various causes (headaches, body aches, arthritis, dysmenorrhea, neuralgia,gout, etc), and for febrile states, aspirin is also useful in the treatment of rheumatic disease, and as an anti-platelet (to thin the blood and prevent blood clots) in coronary (heart) artery and in the deep veins in the legs and pelvis. There have also been articles written in the medical literature postulating reduction in the incidence of colon cancer among those people regularly taking aspirin at a certain dose. Many physicians and patients today take low-dose aspirin (baby aspirin or 81 mg.) daily to reduce the chances of getting a heart attack and stroke by its anti-platelet (blood thinning) action.
Are there other known uses for aspirin?
Aspirin has also been used with success in the treatment of children with Bartter's Syndrome, and also in enhancing the closure of Patent Ductus Arteriosus, an abnormal connection between the aorta (main artery connected to the heart) and the pulmonary artery (to the lungs) in the newborn. If the PDA does not close normally, surgery may be needed to ligate it (close with sutures) before the child starts school.
Is coated aspirin less irritating to the stomach?
Yes, enteric-coated aspirin is much less irritating to the stomach, since the coating allows the aspirin tablet to remain almost intact while in the stomach and travel to the small intestine where the coating and the aspirin dissolve and get absorbed into the blood stream. This minimizes stomach ulcers but does not totally prevent them. Buffered aspirin (one that has anti-acid) also lessens stomach irritation. The bleeding from aspirin ingestion could be serious. This is why anyone interested in taking aspirin must consult a physician before actually taking it.
Why is aspirin being prescribed for heart attack and stroke prevention?
Aspirin, as we stated earlier, thins the blood by preventing platelet aggregation. Platelets are blood component that plays a role in blood thickening or clot formation. When they aggregate (clump together) blood thickens and clots form. Clots tend to clog arteries and veins. When arteries to the heart (coronary) get severely blocked by clots, heart attack occurs, and when this clogging happens to the arteries to the brain, stroke happens. As simple as aspirin, this wonder drug, plays a very vital role in these conditions, together with a change in lifestyle (no smoking, low cholesterol diet, regular exercises, etc.) to maintain a thinner blood condition.
Is aspirin safe for children?
Pediatricians all over the world have for almost 3 decades discontinued prescribing aspirin for children for pain and fever, because aspirin has been implicated in the occurrence of Reye's Syndrome in children following a viral (upper respiratory or gastrointestinal) infection, which syndrome could be fatal. For fever or pain, physicians now prefer to prescribe acetaminophen (like Tylenol) or Ibuprofen, but for some specific illnesses (like Kawasaki Disease, Juvenile Rheumatoid Arthritis, etc.) aspirin is still being used effectively by Pediatricians.
Is aspirin safe for adults who self-medicate?
While occasionally taking two tablets of aspirin for ordinary headache or muscle aches and pains is fairly safe (unless one has a stomach ulcer or a history of ulcer, or is on blood thinner), taking aspirin on a long term basis for prophylaxis or for any condition should only be done under the supervision of a physician.
Aspirin: The Wonder Drug
Aspirin (medically known as acetylsalicylic acid) is a common household medication for pain (analgesic), fever (antipyretic), and inflammation (anti-inflammatory). This "simple" and inexpensive drug is so much underrated and practically taken for granted. Aspirin is really a versatile drug, with a lot of uses, much more than the lay public realizes. Inn the United States alone about 20 tons of aspirin is used each year.
How did aspirin come about?
In 1827, Leroux of France first discovered salicin, an active ingredient in the willow bark, and in 1838, Piria produced salicylic acid from salicin. In 1899, Dreser introduced acetylsalicylic acid (aspirin) into medicine. This has been in popular use since then.
Why is aspirin a Wonder Drug?
Aspirin was a labeled a Miracle Drug or a Wonder Drug when it was first introduced more than 100 years ago, and continues to deserve such a prestigious position in the physician's armamentarium because of its versatility. Other Miracle or Wonder Drugs when they were first introduced include Penicillin, Steroids, Chemo-therapeutic Agents for cancers, and lately, Viagra, for male erectile dysfunction.
How is aspirin versatile?
Besides being used as an analgesic for pain of various causes (headaches, body aches, arthritis, dysmenorrhea, neuralgia,gout, etc), and for febrile states, aspirin is also useful in the treatment of rheumatic disease, and as an anti-platelet (to thin the blood and prevent blood clots) in coronary (heart) artery and in the deep veins in the legs and pelvis. There have also been articles written in the medical literature postulating reduction in the incidence of colon cancer among those people regularly taking aspirin at a certain dose. Many physicians and patients today take low-dose aspirin (baby aspirin or 81 mg.) daily to reduce the chances of getting a heart attack and stroke by its anti-platelet (blood thinning) action.
Are there other known uses for aspirin?
Aspirin has also been used with success in the treatment of children with Bartter's Syndrome, and also in enhancing the closure of Patent Ductus Arteriosus, an abnormal connection between the aorta (main artery connected to the heart) and the pulmonary artery (to the lungs) in the newborn. If the PDA does not close normally, surgery may be needed to ligate it (close with sutures) before the child starts school.
Is coated aspirin less irritating to the stomach?
Yes, enteric-coated aspirin is much less irritating to the stomach, since the coating allows the aspirin tablet to remain almost intact while in the stomach and travel to the small intestine where the coating and the aspirin dissolve and get absorbed into the blood stream. This minimizes stomach ulcers but does not totally prevent them. Buffered aspirin (one that has anti-acid) also lessens stomach irritation. The bleeding from aspirin ingestion could be serious. This is why anyone interested in taking aspirin must consult a physician before actually taking it.
Why is aspirin being prescribed for heart attack and stroke prevention?
Aspirin, as we stated earlier, thins the blood by preventing platelet aggregation. Platelets are blood component that plays a role in blood thickening or clot formation. When they aggregate (clump together) blood thickens and clots form. Clots tend to clog arteries and veins. When arteries to the heart (coronary) get severely blocked by clots, heart attack occurs, and when this clogging happens to the arteries to the brain, stroke happens. As simple as aspirin, this wonder drug, plays a very vital role in these conditions, together with a change in lifestyle (no smoking, low cholesterol diet, regular exercises, etc.) to maintain a thinner blood condition.
Is aspirin safe for children?
Pediatricians all over the world have for almost 3 decades discontinued prescribing aspirin for children for pain and fever, because aspirin has been implicated in the occurrence of Reye's Syndrome in children following a viral (upper respiratory or gastrointestinal) infection, which syndrome could be fatal. For fever or pain, physicians now prefer to prescribe acetaminophen (like Tylenol) or Ibuprofen, but for some specific illnesses (like Kawasaki Disease, Juvenile Rheumatoid Arthritis, etc.) aspirin is still being used effectively by Pediatricians.
Is aspirin safe for adults who self-medicate?
While occasionally taking two tablets of aspirin for ordinary headache or muscle aches and pains is fairly safe (unless one has a stomach ulcer or a history of ulcer, or is on blood thinner), taking aspirin on a long term basis for prophylaxis or for any condition should only be done under the supervision of a physician.
Cause of KD remains a mystery
Cause of Kawasaki disease remains a mystery
Cause of Kawasaki disease remains a mystery
Recent evidence suggests an infectious agent, although to date no organism has been found
Monday, February 02, 2009
Staten Island AdvanceSTATEN ISLAND, N.Y. -- Kawasaki disease is an acute, self-limited disease of unknown cause that has a tendency to affect the coronary arteries (the arteries supplying the heart). It predominantly affects infants and children. Kawasaki disease is an illness that involves the skin, mouth, and lymph nodes. It is associated with high fevers that last for five or more days.
The disease was first described in Japan in 1967 by Tomisaku Kawasaki; it is now known to occur in the Americas, Europe, and Asia in children of all races. Kawasaki disease is markedly more common in Japan and children of Japanese ethnicity, having an annual incidence of 150 cases per 100,000 children younger than 5 years of age. Recurrence rates approach 3 percent. About 1 percent of those with the disease have a family history. The risk of occurrence in twins is approximately 13 percent. These statistics suggest that genetic predisposition may play a role in who gets the disease. The cause of Kawasaki disease remains a mystery. All the recent evidence suggests an infectious agent, although to date no organism has been found. This disease occurs in phases. The first phase, which can last for up to two weeks, usually involves a persistent fever higher than 104 degrees Fahrenheit and lasts for at least five days. The second phase occurs two weeks after the fever begins, and the skin of the hands and feet may begin to peel in large pieces. The child may also experience joint pain, diarrhea, vomiting, or abdominal pain. Kawasaki disease is a generalized inflammation of blood vessels, affecting all blood vessels throughout the body, but preferentially affects the coronary arteries. DIAGNOSITC CRITERIA
Cause of Kawasaki disease remains a mystery - Page 2
Recent evidence suggests an infectious agent, although to date no organism has been foundThe diagnostic criteria for Kawasaki disease requires fever for at least five days; and four of the five following criteria: Bilateral Conjunctiva injection (blood-shot eyes). Changes of the mucous membranes of the upper respiratory tract: red pharynx, fissured or cracked lips, strawberry appearing tongue. Rash. Changes to the extremities (swelling, redness, skin peeling). Enlarged lymph nodes in the neck region. A sonogram (echocardiogram) is done as soon as the diagnosis is entertained to rule out any inflammation of the heart and to check for aneurysm formation of the coronary arteries. It is then done periodically to monitor the condition. If suspected of having this disease, treatment is started immediately with aspirin, and intravenous immunoglobulin. These both have anti-inflammatory effects. The sooner these medications are started, the less likely complications may occur. If a child is diagnosed with an aneurysm, aspirin is continued indefinitely to prevent rupture of the aneurysm or clot formation. Coronary artery aneurysms occur in 20 to 25 percent of untreated children. Resolution of aneurysms occurs in one to two years for approximately 50 percent of patients. Myocardial infarction (heart attack) caused by a clot in an abnormal coronary artery is the principal cause of death. The greatest risk occurs in the first year after diagnosis. INCOMPLETE FORM Recent evidence suggests there may be an incomplete form of Kawasaki disease occurring in some individuals. Some patients do not fulfill the clinical criteria for the disease and are diagnosed based on echocardiographic findings of coronary artery anomalies. Therefore, strict adherence to the diagnostic criteria may in fact miss some cases. So when should incomplete Kawasaki disease be considered? If a child has unexplained fever for five or more days associated with two or three of the diagnostic criteria mentioned above. Other diseases that may present in a similar manor include: measles, strep throat and drug hypersensitivity reactions. Kawasaki disease can be quite devastating. It is imperative to seek medical attention as soon as possible, as the earlier it is diagnosed the better the outcome. This column is provided by the Richmond County Medical Society. Dr. Messo is the immediate past president of Society. He maintains a private practice in Eltingville and is director of internal medicine/pediatric education at Richmond University Medical Center and an adjunct clinical professor at Touro College of Osteopathic Medicine in Harlem.
Cause of Kawasaki disease remains a mystery
Recent evidence suggests an infectious agent, although to date no organism has been found
Monday, February 02, 2009
Staten Island AdvanceSTATEN ISLAND, N.Y. -- Kawasaki disease is an acute, self-limited disease of unknown cause that has a tendency to affect the coronary arteries (the arteries supplying the heart). It predominantly affects infants and children. Kawasaki disease is an illness that involves the skin, mouth, and lymph nodes. It is associated with high fevers that last for five or more days.
The disease was first described in Japan in 1967 by Tomisaku Kawasaki; it is now known to occur in the Americas, Europe, and Asia in children of all races. Kawasaki disease is markedly more common in Japan and children of Japanese ethnicity, having an annual incidence of 150 cases per 100,000 children younger than 5 years of age. Recurrence rates approach 3 percent. About 1 percent of those with the disease have a family history. The risk of occurrence in twins is approximately 13 percent. These statistics suggest that genetic predisposition may play a role in who gets the disease. The cause of Kawasaki disease remains a mystery. All the recent evidence suggests an infectious agent, although to date no organism has been found. This disease occurs in phases. The first phase, which can last for up to two weeks, usually involves a persistent fever higher than 104 degrees Fahrenheit and lasts for at least five days. The second phase occurs two weeks after the fever begins, and the skin of the hands and feet may begin to peel in large pieces. The child may also experience joint pain, diarrhea, vomiting, or abdominal pain. Kawasaki disease is a generalized inflammation of blood vessels, affecting all blood vessels throughout the body, but preferentially affects the coronary arteries. DIAGNOSITC CRITERIA
Cause of Kawasaki disease remains a mystery - Page 2
Recent evidence suggests an infectious agent, although to date no organism has been foundThe diagnostic criteria for Kawasaki disease requires fever for at least five days; and four of the five following criteria: Bilateral Conjunctiva injection (blood-shot eyes). Changes of the mucous membranes of the upper respiratory tract: red pharynx, fissured or cracked lips, strawberry appearing tongue. Rash. Changes to the extremities (swelling, redness, skin peeling). Enlarged lymph nodes in the neck region. A sonogram (echocardiogram) is done as soon as the diagnosis is entertained to rule out any inflammation of the heart and to check for aneurysm formation of the coronary arteries. It is then done periodically to monitor the condition. If suspected of having this disease, treatment is started immediately with aspirin, and intravenous immunoglobulin. These both have anti-inflammatory effects. The sooner these medications are started, the less likely complications may occur. If a child is diagnosed with an aneurysm, aspirin is continued indefinitely to prevent rupture of the aneurysm or clot formation. Coronary artery aneurysms occur in 20 to 25 percent of untreated children. Resolution of aneurysms occurs in one to two years for approximately 50 percent of patients. Myocardial infarction (heart attack) caused by a clot in an abnormal coronary artery is the principal cause of death. The greatest risk occurs in the first year after diagnosis. INCOMPLETE FORM Recent evidence suggests there may be an incomplete form of Kawasaki disease occurring in some individuals. Some patients do not fulfill the clinical criteria for the disease and are diagnosed based on echocardiographic findings of coronary artery anomalies. Therefore, strict adherence to the diagnostic criteria may in fact miss some cases. So when should incomplete Kawasaki disease be considered? If a child has unexplained fever for five or more days associated with two or three of the diagnostic criteria mentioned above. Other diseases that may present in a similar manor include: measles, strep throat and drug hypersensitivity reactions. Kawasaki disease can be quite devastating. It is imperative to seek medical attention as soon as possible, as the earlier it is diagnosed the better the outcome. This column is provided by the Richmond County Medical Society. Dr. Messo is the immediate past president of Society. He maintains a private practice in Eltingville and is director of internal medicine/pediatric education at Richmond University Medical Center and an adjunct clinical professor at Touro College of Osteopathic Medicine in Harlem.
A world of genetic research
A world of genetic research
A world of genetic research
Last Updated: March 11. 2009 1:12AM UAE / March 10. 2009 9:12PM GMT..
The death of Jett Travolta, third from left, brought the rare childhoodcondition known as Kawasaki disease to the attention of the public. APKawasakidisease, a rare autoimmune illness that mostly affects young children,was in the news recently in two different stories. In the first case,the disease was linked with Jett Travolta, son of the actor JohnTravolta. Jett died on Jan 2 after he had a seizure. He had apparentlybeen ill for some time and his parents said he had Kawasaki disease.Itbrought the condition to the attention of the public, and was followedby news of research in Australia into how the disease behaves.ProfDavid Burgner from the University of Western Australia co-led a teamthat studied the entire human genome in an attempt to find new genesinvolved in making some children more susceptible to the disease. Itwas first described by Dr Tomisaku Kawasaki in 1967, and although thecondition is treatable, little has been learnt since about its causes.Itpresents itself as an inflammatory condition. It affects several organsincluding the mucus membranes, the walls of blood vessels and lymphnodes. Its many symptoms include fever, rashes, swollen feet and hands,conjunctivitis and swollen lymph nodes. Children aged six months tofive years seem particularly susceptible. It can be fatal, but only onein 1,000 cases result in death. Although it is rare, Prof Burgnerexplained why it was important to tackle the illness.“Kawasakidisease is an important and serious illness of preschool children,” hesaid. “Importantly it is the only childhood illness that damages thecoronary arteries, in one quarter of untreated children and five to 10per cent of treated children. This coronary damage may have long-termhealth implications, including death, angina and heart attacks inchildhood and the need for coronary bypass surgery and even, rarely,heart transplant in childhood.”In the research study ProfBurgner’s team considered genetic variation among 900 cases of thedisease found in children from the US, Singapore, the Netherlands, theUK and Australia. The findings identified genes that may increase thesusceptibility of some children to the disease, but further research isneeded.“Kawasaki disease is thought to be an abnormalimmunological reaction to an unknown infectious trigger. There is astrong genetic component, so genes are important in determiningsusceptibility, as we believe all children are likely to be exposed tothe trigger,” Prof Burgner said.“Our study is the firstpublished that has investigated the entire human genome looking forimportant associated genes; most studies focus on a few candidategenes. We have identified at least eight new genes that have notpreviously been described in Kawasaki disease and some of them seem tofunction together. Some of the genes are involved in cardiovascularhealth – the function of blood vessels and in control of the immunesystem.”Identifying eight genes expands the scope of futureresearch considerably. However, studying the entire human genome is notthe biggest problem facing the researchers.“The main issue isrecruiting enough cases of Kawasaki disease, which is why we embarkedon an international study. Ideally we need up to 5,000 cases to covermore of the human genome and find more of the important genes. We werevery lucky to work with the Genome Institute of Singapore, who are oneof the top genomics facilities in the world – this made the actualgenetic laboratory work and analysis possible.”Dr BrianMcCrindle, a cardiologist from the Hospital for Sick Children inToronto, Canada, gave a talk on Kawasaki disease at the Arab HealthCongress in Dubai in January. He believes the new findings offerpotential for future research.“The research is very preliminaryand needs to be verified in other people’s work, but there are somepromising leads,” he said. “Kawasaki disease can look like otherdiseases and sometimes it doesn’t have typical presentation,” he said.Raisingawareness is vital because the disease can be misdiagnosed, butdeveloping a cure for any rare disease is difficult. However, ProfBurgner believes the identification of eight new genes will help withthe treatment process.“We hope that our work will contribute tothe development of a diagnostic test and better treatment within thenext few years, but we are not there yet,” he said.“At themoment doctors make a clinical diagnosis, based on the rash, fever etcetera, and there is no blood test that really helps. We urgently needa diagnostic test so children are treated earlier and Kawasaki diseaseis not missed, so reducing the long-term heart problems.”Findinga cure remains a possibility, though. “In Japan, for example, where onein 150 children suffer KD, it may be realistic to develop a vaccine,especially if Kawasaki disease increases the risk of atherosclerosis inlater life – this is an unknown area at present,” Prof Burgner said.Healso believes that the method used in his research of analysing theentire human genome will be adopted as the way forward in studyingother infectious diseases.“Genes are known to be important indetermining susceptibility to infection so it is a proven and excitingmethod to try and identify the important genes that make people more orless likely to get infection and once they have it, more or less likelyto survive. This should lead to better prevention and treatment. Isuspect tuberculosis, HIV, hepatitis B and C and malaria will be thelikely diseases that we will see these sorts of studies in very soon,”he said.For the moment, however, Prof Burgner is keen tocontinue to develop the research path he is pursuing with Kawasakidisease. There is also the possibility of collaborating with medicalprofessionals in the Middle East. “We are expanding thecollaborative genetics group to include Asian and other populations,aiming to work together as a scientific community to crack thismysterious disease. We would very much like to hear from anyone in yourregion who has an interest in Kawasaki disease and sees many patients,”he said.Peter Donnelly is a science correspondent for the life science division at IIR Middle East
A world of genetic research
Last Updated: March 11. 2009 1:12AM UAE / March 10. 2009 9:12PM GMT..
The death of Jett Travolta, third from left, brought the rare childhoodcondition known as Kawasaki disease to the attention of the public. APKawasakidisease, a rare autoimmune illness that mostly affects young children,was in the news recently in two different stories. In the first case,the disease was linked with Jett Travolta, son of the actor JohnTravolta. Jett died on Jan 2 after he had a seizure. He had apparentlybeen ill for some time and his parents said he had Kawasaki disease.Itbrought the condition to the attention of the public, and was followedby news of research in Australia into how the disease behaves.ProfDavid Burgner from the University of Western Australia co-led a teamthat studied the entire human genome in an attempt to find new genesinvolved in making some children more susceptible to the disease. Itwas first described by Dr Tomisaku Kawasaki in 1967, and although thecondition is treatable, little has been learnt since about its causes.Itpresents itself as an inflammatory condition. It affects several organsincluding the mucus membranes, the walls of blood vessels and lymphnodes. Its many symptoms include fever, rashes, swollen feet and hands,conjunctivitis and swollen lymph nodes. Children aged six months tofive years seem particularly susceptible. It can be fatal, but only onein 1,000 cases result in death. Although it is rare, Prof Burgnerexplained why it was important to tackle the illness.“Kawasakidisease is an important and serious illness of preschool children,” hesaid. “Importantly it is the only childhood illness that damages thecoronary arteries, in one quarter of untreated children and five to 10per cent of treated children. This coronary damage may have long-termhealth implications, including death, angina and heart attacks inchildhood and the need for coronary bypass surgery and even, rarely,heart transplant in childhood.”In the research study ProfBurgner’s team considered genetic variation among 900 cases of thedisease found in children from the US, Singapore, the Netherlands, theUK and Australia. The findings identified genes that may increase thesusceptibility of some children to the disease, but further research isneeded.“Kawasaki disease is thought to be an abnormalimmunological reaction to an unknown infectious trigger. There is astrong genetic component, so genes are important in determiningsusceptibility, as we believe all children are likely to be exposed tothe trigger,” Prof Burgner said.“Our study is the firstpublished that has investigated the entire human genome looking forimportant associated genes; most studies focus on a few candidategenes. We have identified at least eight new genes that have notpreviously been described in Kawasaki disease and some of them seem tofunction together. Some of the genes are involved in cardiovascularhealth – the function of blood vessels and in control of the immunesystem.”Identifying eight genes expands the scope of futureresearch considerably. However, studying the entire human genome is notthe biggest problem facing the researchers.“The main issue isrecruiting enough cases of Kawasaki disease, which is why we embarkedon an international study. Ideally we need up to 5,000 cases to covermore of the human genome and find more of the important genes. We werevery lucky to work with the Genome Institute of Singapore, who are oneof the top genomics facilities in the world – this made the actualgenetic laboratory work and analysis possible.”Dr BrianMcCrindle, a cardiologist from the Hospital for Sick Children inToronto, Canada, gave a talk on Kawasaki disease at the Arab HealthCongress in Dubai in January. He believes the new findings offerpotential for future research.“The research is very preliminaryand needs to be verified in other people’s work, but there are somepromising leads,” he said. “Kawasaki disease can look like otherdiseases and sometimes it doesn’t have typical presentation,” he said.Raisingawareness is vital because the disease can be misdiagnosed, butdeveloping a cure for any rare disease is difficult. However, ProfBurgner believes the identification of eight new genes will help withthe treatment process.“We hope that our work will contribute tothe development of a diagnostic test and better treatment within thenext few years, but we are not there yet,” he said.“At themoment doctors make a clinical diagnosis, based on the rash, fever etcetera, and there is no blood test that really helps. We urgently needa diagnostic test so children are treated earlier and Kawasaki diseaseis not missed, so reducing the long-term heart problems.”Findinga cure remains a possibility, though. “In Japan, for example, where onein 150 children suffer KD, it may be realistic to develop a vaccine,especially if Kawasaki disease increases the risk of atherosclerosis inlater life – this is an unknown area at present,” Prof Burgner said.Healso believes that the method used in his research of analysing theentire human genome will be adopted as the way forward in studyingother infectious diseases.“Genes are known to be important indetermining susceptibility to infection so it is a proven and excitingmethod to try and identify the important genes that make people more orless likely to get infection and once they have it, more or less likelyto survive. This should lead to better prevention and treatment. Isuspect tuberculosis, HIV, hepatitis B and C and malaria will be thelikely diseases that we will see these sorts of studies in very soon,”he said.For the moment, however, Prof Burgner is keen tocontinue to develop the research path he is pursuing with Kawasakidisease. There is also the possibility of collaborating with medicalprofessionals in the Middle East. “We are expanding thecollaborative genetics group to include Asian and other populations,aiming to work together as a scientific community to crack thismysterious disease. We would very much like to hear from anyone in yourregion who has an interest in Kawasaki disease and sees many patients,”he said.Peter Donnelly is a science correspondent for the life science division at IIR Middle East
KD an overview
Kawasaki disease: an overview
Current Opinion in Infectious Diseases:
June 2008 - Volume 21 - Issue 3 - p 263-270
doi: 10.1097/QCO.0b013e3282fbf9cd
Paediatric and neonatal infections: Edited by Paul T. Heath
Kawasaki disease: an overview
Pinna, Georgia S; Kafetzis, Dimitris A; Tselkas, Orestis I; Skevaki, Chrysanthi L
Abstract
Purpose of review: Kawasaki disease is an acute, self-limited vasculitis of childhood. The increasing frequency of the disease as well as the deficiency of specific diagnostic means renders its diagnosis and treatment an area of intense investigation. The purpose of this review is to summarize all the known features of Kawasaki disease and also give an insight to the latest findings.
Recent findings: Kawasaki disease is one of the leading causes of acquired heart disease in children while its cause remains essentially unknown. Viruses, bacterial conventional as well as superantigens, and genetic polymorphisms have been implicated in the etiology of the disease. Markers of inflammation, such as CCL2 and CCXCL10, contribute to the pathology and the diagnosis of Kawasaki disease. Intravenous administration of immunoglobulin remains the mainstay of therapy for Kawasaki disease. Nevertheless, forms of the disease refractory to intravenous administration of immunoglobulin therapy may respond to aspirin, corticosteroids, cyclophosphamide, and/or plasmapheresis.
Summary: The present review covers evidence regarding the history of Kawasaki disease, the epidemiology, etiology, pathology, genetic influences, and long-term sequela. It also includes an evaluation of contemporary diagnostic techniques and optimal therapeutic approaches with an emphasis on recent publications.
Current Opinion in Infectious Diseases:
June 2008 - Volume 21 - Issue 3 - p 263-270
doi: 10.1097/QCO.0b013e3282fbf9cd
Paediatric and neonatal infections: Edited by Paul T. Heath
Kawasaki disease: an overview
Pinna, Georgia S; Kafetzis, Dimitris A; Tselkas, Orestis I; Skevaki, Chrysanthi L
Abstract
Purpose of review: Kawasaki disease is an acute, self-limited vasculitis of childhood. The increasing frequency of the disease as well as the deficiency of specific diagnostic means renders its diagnosis and treatment an area of intense investigation. The purpose of this review is to summarize all the known features of Kawasaki disease and also give an insight to the latest findings.
Recent findings: Kawasaki disease is one of the leading causes of acquired heart disease in children while its cause remains essentially unknown. Viruses, bacterial conventional as well as superantigens, and genetic polymorphisms have been implicated in the etiology of the disease. Markers of inflammation, such as CCL2 and CCXCL10, contribute to the pathology and the diagnosis of Kawasaki disease. Intravenous administration of immunoglobulin remains the mainstay of therapy for Kawasaki disease. Nevertheless, forms of the disease refractory to intravenous administration of immunoglobulin therapy may respond to aspirin, corticosteroids, cyclophosphamide, and/or plasmapheresis.
Summary: The present review covers evidence regarding the history of Kawasaki disease, the epidemiology, etiology, pathology, genetic influences, and long-term sequela. It also includes an evaluation of contemporary diagnostic techniques and optimal therapeutic approaches with an emphasis on recent publications.
KD Long term health
Article on KD long term health
PEDIATRICS Vol. 111 No. 3 March 2003, pp. 579-583....
Physical and Psychosocial Health in Children Who Have Had Kawasaki Disease ....Annette L. Baker, MSN, PNP*.., ..Kimberlee Gauvreau, ScD*.., ..Jane W. Newburger, MD, MPH*.., ..Robert P. Sundel, MD*.., ..David R. Fulton, MD*.. and ..Kathy J. Jenkins, MD, MPH*.. * From the Department of Cardiology, Children’s Hospital, Boston, Massachusetts; and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts ....-->.. Objective. The purpose of this study was to examine the physical and psychosocial well-being of children who have had Kawasaki disease (KD), including the influence of coronary artery status on health and health perceptions. Methods. The Child Health Questionnaire (CHQ) measures overall physical and psychosocial well-being in children 5 to 18 years. To study the long-term impact of KD on overall health status, we mailed the CHQ to patients without a history of coronary artery abnormalities (normal group), with regressed aneurysms (regressed group), with current coronary aneurysms <8 aneurysms =" src=" border="0">8 mm (giant group). Results. Of 201 questionnaires mailed, 174 were delivered and 110 (63%) were completed. Median age (range) at completion was 10.5 years (5.1–17.9 years) and at illness onset was 3.1 years (0.2–12 years). There were no significant differences in psychosocial summary scores in any of the Kawasaki groups when compared with the US population sample. Physical summary scores were also similar to the US population sample in the normal coronary, mild-moderate aneurysm, and regressed aneurysm groups. However, the giant aneurysm group had significantly lower physical summary scores compared with the US population sample. Among subscales, general health perceptions in the KD groups were lower than in the US population sample, reaching statistical significance in all but the mild to moderate aneurysm group. In addition, parents whose children have had KD reported a higher proportion of anxiety issues, allergies, and orthopedic/bone/joint issues in their children than did the general US population sample. We did not find any difference in the incidence of attentional, behavioral, or learning issues when compared with the US population sample. Conclusions. KD patients without coronary artery aneurysms were similar to the general population in their general physical and psychosocial health. However, the parents of children in all KD groups reported lower general health perceptions than parents in the US population sample, suggesting that long-term concerns about their children’s health exist regardless of overall health status. In addition, children with giant coronary artery aneurysms had lower overall physical summary scores. Key Words: Kawasaki disease • Child Health Questionnaire • functional health status • quality of life Abbreviations: KD, Kawasaki disease • CHQ, Child Health Questionnaire • JRA, juvenile rheumatoid arthritis • ADHD, attention-deficit/hyperactivity disorder
Received for publication May 7, 2002; Accepted Sep 11, 2002.
PEDIATRICS Vol. 111 No. 3 March 2003, pp. 579-583....
Physical and Psychosocial Health in Children Who Have Had Kawasaki Disease ....Annette L. Baker, MSN, PNP*.., ..Kimberlee Gauvreau, ScD*.., ..Jane W. Newburger, MD, MPH*.., ..Robert P. Sundel, MD*.., ..David R. Fulton, MD*.. and ..Kathy J. Jenkins, MD, MPH*.. * From the Department of Cardiology, Children’s Hospital, Boston, Massachusetts; and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts ....-->.. Objective. The purpose of this study was to examine the physical and psychosocial well-being of children who have had Kawasaki disease (KD), including the influence of coronary artery status on health and health perceptions. Methods. The Child Health Questionnaire (CHQ) measures overall physical and psychosocial well-being in children 5 to 18 years. To study the long-term impact of KD on overall health status, we mailed the CHQ to patients without a history of coronary artery abnormalities (normal group), with regressed aneurysms (regressed group), with current coronary aneurysms <8 aneurysms =" src=" border="0">8 mm (giant group). Results. Of 201 questionnaires mailed, 174 were delivered and 110 (63%) were completed. Median age (range) at completion was 10.5 years (5.1–17.9 years) and at illness onset was 3.1 years (0.2–12 years). There were no significant differences in psychosocial summary scores in any of the Kawasaki groups when compared with the US population sample. Physical summary scores were also similar to the US population sample in the normal coronary, mild-moderate aneurysm, and regressed aneurysm groups. However, the giant aneurysm group had significantly lower physical summary scores compared with the US population sample. Among subscales, general health perceptions in the KD groups were lower than in the US population sample, reaching statistical significance in all but the mild to moderate aneurysm group. In addition, parents whose children have had KD reported a higher proportion of anxiety issues, allergies, and orthopedic/bone/joint issues in their children than did the general US population sample. We did not find any difference in the incidence of attentional, behavioral, or learning issues when compared with the US population sample. Conclusions. KD patients without coronary artery aneurysms were similar to the general population in their general physical and psychosocial health. However, the parents of children in all KD groups reported lower general health perceptions than parents in the US population sample, suggesting that long-term concerns about their children’s health exist regardless of overall health status. In addition, children with giant coronary artery aneurysms had lower overall physical summary scores. Key Words: Kawasaki disease • Child Health Questionnaire • functional health status • quality of life Abbreviations: KD, Kawasaki disease • CHQ, Child Health Questionnaire • JRA, juvenile rheumatoid arthritis • ADHD, attention-deficit/hyperactivity disorder
Received for publication May 7, 2002; Accepted Sep 11, 2002.
KD INFO
Surveys on children with Kawasaki disease in Japan
Mortality among children with Kawasaki disease in Japan
Article Abstract:The risk of dying for boys with Kawasaki disease may be twice as high as that of healthy boys the same age, and higher than that of girls with Kawasaki disease. Kawasaki disease is a disorder that affects mainly infants and toddlers; causes inflammation of the blood and lymphatic vessels throughout the body. Of 4,608 Japanese children with Kawasaki disease who could be followed, 13 died during the 18-month study: 10 boys and three girls. The number of deaths among these children was higher than the expected number for healthy children the same age. The number of boys with Kawasaki disease who died was approximately two times higher than the expected number for age-matched healthy boys, while the number of deaths among girls with Kawasaki disease was approximately the same as for age-matched healthy girls. The number of deaths during the acute phase of the disease, or the first two months after its onset, was much higher than for comparable healthy children. After the acute phase, the number of deaths was approximately equal to the expected number.
author: Nakamura, Yosikazu, Yanagawa, Hiroshi, Kawasaki, Tomisaku
Publisher: Massachusetts Medical SocietyPublication Name: The New England Journal of MedicineSubject: HealthISSN: 0028-4793Year: 1992
Serum concentrations of total bile acids in patients with acute Kawasaki syndrome
Article Abstract:The association between the increase in total bile acid (TBA) levels seen in some children with Kawasaki syndrome (KS) and other measurements of liver function is unclear. The liver produces the bile acids that aid in the digestion of fats. Researchers evaluated the blood levels of TBA, alanine aminotransferase (ALT), total bilirubin (TBil), alkaline phosphatase (ALP), and C-reactive protein (CRP) in 71 children aged 2 months to 8 years in the initial phase of KS. They repeated these blood tests in 29 of these patients during their recovery. Twenty-two percent of the 71 patients had increased TBA levels during the initial phase of KS. TBil and ALT levels were normal in some of these patients with increased TBA levels. There was no association between TBAs and any of the other liver function tests in 10 patients who also had heart complications. TBA levels returned to normal in all but three patients evaluated during the recovery phase.
author: Kato, Hirohisa, Kimura, Akihiko, Inoue, Osamu
Publisher: American Medical AssociationPublication Name: Archives of Pediatrics & Adolescent MedicineSubject: HealthISSN: 1072-4710Year: 1996
Results of 12 nationwide epidemiological incidence surveys of Kawasaki disease in Japan
Article Abstract:The frequency and distribution of Kawasaki disease in Japan indicates that the disease may not be caused by a virus. Researchers reviewed hospital surveys of Kawasaki disease conducted at two-year intervals between 1970 and 1992. The number of patients with the disease rapidly increased from 1970 to 1986 and remained stable at approximately 5,000 per year thereafter. Outbreaks occurred in 1979, 1982, and 1986 during which the rate of sibling cases increased. Males and children younger than one year of age had higher rates of Kawasaki disease and heart complications than other children. The recurrence rate ranged between .3% and 5% and increased slightly following outbreaks. The death rate decreased from 1% in 1974 to .04% in 1992. Corticosteroid treatment decreased significantly from 1975 to 1990 while the use of immune globulins increased sharply from 1982 to 1992.
author: Nakamura, Yosikazu, Yanagawa, Hiroshi, Kawasaki, Tomisaku, Yashiro, Mayumi, Kato, Hirohisa
Publisher: American Medical AssociationPublication Name: Archives of Pediatrics & Adolescent MedicineSubject: HealthISSN: 1072-4710Year: 1995
Mortality among children with Kawasaki disease in Japan
Article Abstract:The risk of dying for boys with Kawasaki disease may be twice as high as that of healthy boys the same age, and higher than that of girls with Kawasaki disease. Kawasaki disease is a disorder that affects mainly infants and toddlers; causes inflammation of the blood and lymphatic vessels throughout the body. Of 4,608 Japanese children with Kawasaki disease who could be followed, 13 died during the 18-month study: 10 boys and three girls. The number of deaths among these children was higher than the expected number for healthy children the same age. The number of boys with Kawasaki disease who died was approximately two times higher than the expected number for age-matched healthy boys, while the number of deaths among girls with Kawasaki disease was approximately the same as for age-matched healthy girls. The number of deaths during the acute phase of the disease, or the first two months after its onset, was much higher than for comparable healthy children. After the acute phase, the number of deaths was approximately equal to the expected number.
author: Nakamura, Yosikazu, Yanagawa, Hiroshi, Kawasaki, Tomisaku
Publisher: Massachusetts Medical SocietyPublication Name: The New England Journal of MedicineSubject: HealthISSN: 0028-4793Year: 1992
Serum concentrations of total bile acids in patients with acute Kawasaki syndrome
Article Abstract:The association between the increase in total bile acid (TBA) levels seen in some children with Kawasaki syndrome (KS) and other measurements of liver function is unclear. The liver produces the bile acids that aid in the digestion of fats. Researchers evaluated the blood levels of TBA, alanine aminotransferase (ALT), total bilirubin (TBil), alkaline phosphatase (ALP), and C-reactive protein (CRP) in 71 children aged 2 months to 8 years in the initial phase of KS. They repeated these blood tests in 29 of these patients during their recovery. Twenty-two percent of the 71 patients had increased TBA levels during the initial phase of KS. TBil and ALT levels were normal in some of these patients with increased TBA levels. There was no association between TBAs and any of the other liver function tests in 10 patients who also had heart complications. TBA levels returned to normal in all but three patients evaluated during the recovery phase.
author: Kato, Hirohisa, Kimura, Akihiko, Inoue, Osamu
Publisher: American Medical AssociationPublication Name: Archives of Pediatrics & Adolescent MedicineSubject: HealthISSN: 1072-4710Year: 1996
Results of 12 nationwide epidemiological incidence surveys of Kawasaki disease in Japan
Article Abstract:The frequency and distribution of Kawasaki disease in Japan indicates that the disease may not be caused by a virus. Researchers reviewed hospital surveys of Kawasaki disease conducted at two-year intervals between 1970 and 1992. The number of patients with the disease rapidly increased from 1970 to 1986 and remained stable at approximately 5,000 per year thereafter. Outbreaks occurred in 1979, 1982, and 1986 during which the rate of sibling cases increased. Males and children younger than one year of age had higher rates of Kawasaki disease and heart complications than other children. The recurrence rate ranged between .3% and 5% and increased slightly following outbreaks. The death rate decreased from 1% in 1974 to .04% in 1992. Corticosteroid treatment decreased significantly from 1975 to 1990 while the use of immune globulins increased sharply from 1982 to 1992.
author: Nakamura, Yosikazu, Yanagawa, Hiroshi, Kawasaki, Tomisaku, Yashiro, Mayumi, Kato, Hirohisa
Publisher: American Medical AssociationPublication Name: Archives of Pediatrics & Adolescent MedicineSubject: HealthISSN: 1072-4710Year: 1995
Aussie professor discovers danger gene for deadly Kawasaki disease
Aussie professor discovers danger gene for deadly Kawasaki disease
Aussie professor discovers danger gene for deadly Kawasaki disease
Posted Sat Jan 10, 2009 12:03pm AEDT
The researchers identified genes which could make some children more susceptible. (ABC News)......
An Australian researcher says he has made a breakthrough which could lead to a diagnostic test and better treatment for the potentially fatal Kawasaki disease.The illness is an inflammatory condition in young children that can damage blood vessels.There are up to 200 cases in Australia each year.A team of researchers led by University of Western Australia Professor David Burgner studied almost 900 cases around the world and has identified genes which could make some children more susceptible.Lily Allen was diagnosed with Kawasaki disease before she was two months old.She is now fully recovered, but Lily's mother Amanda says they had to wait several agonising days before a diagnosis could be made."That was the hard thing, we knew nothing of it and it just took so long to actually diagnose it, which was hard," she said. "It emotionally and physically takes its toll on you. You wonder why your baby's so sick and her heart rate was at 180 at rest, so she was constantly in pain and having trouble breathing."Named after the professor who first described it, Kawasaki disease usually affects children aged from six months to four years.The symptoms include fever, rash, swollen hands and feet, and peeling skin.Kawasaki disease also inflames blood vessels and can cause permanent damage to the heart.Professor Burgner says the disease can be difficult to diagnose."It's often mistaken for Measles or severe infections, scarlet fever or even sometimes meningitis," he said. "So this is a mysterious but very serious disease of young children."Like many diseases we think that genetics plays a major role in deciding or determining who actually develops Kawasaki disease. "And we think this because if Japanese children move to America which has a relatively low rate, their risk remains as high as it would be if they were in Japan. "And the risk of brothers and sisters who have had Kawasaki disease is about 10 times the risk of the general population."So we think that genes are going to be important in determining who actually develops Kawasaki disease when they're exposed to whatever it is that's triggering this illness," he added.Professor Burgner says the findings are an important first step in understanding the disease."Ultimately we'd like to develop a diagnostic test, that's really what the paediatricians are crying out for - a bedside or a diagnostic test for Kawasaki disease, because it's a very difficult diagnosis to make sometimes," he said. "We need better treatment because our best treatment actually fails in 5 to 10 per cent of cases to prevent damage to the heart. "It's not inconceivable. In the future we may be able to develop a vaccine to prevent Kawasaki disease and maybe that will have some impact on future risk of heart attack and things like that.Based on report by David Weber for PM Friday january 9, 2009.
Aussie professor discovers danger gene for deadly Kawasaki disease
Posted Sat Jan 10, 2009 12:03pm AEDT
The researchers identified genes which could make some children more susceptible. (ABC News)......
An Australian researcher says he has made a breakthrough which could lead to a diagnostic test and better treatment for the potentially fatal Kawasaki disease.The illness is an inflammatory condition in young children that can damage blood vessels.There are up to 200 cases in Australia each year.A team of researchers led by University of Western Australia Professor David Burgner studied almost 900 cases around the world and has identified genes which could make some children more susceptible.Lily Allen was diagnosed with Kawasaki disease before she was two months old.She is now fully recovered, but Lily's mother Amanda says they had to wait several agonising days before a diagnosis could be made."That was the hard thing, we knew nothing of it and it just took so long to actually diagnose it, which was hard," she said. "It emotionally and physically takes its toll on you. You wonder why your baby's so sick and her heart rate was at 180 at rest, so she was constantly in pain and having trouble breathing."Named after the professor who first described it, Kawasaki disease usually affects children aged from six months to four years.The symptoms include fever, rash, swollen hands and feet, and peeling skin.Kawasaki disease also inflames blood vessels and can cause permanent damage to the heart.Professor Burgner says the disease can be difficult to diagnose."It's often mistaken for Measles or severe infections, scarlet fever or even sometimes meningitis," he said. "So this is a mysterious but very serious disease of young children."Like many diseases we think that genetics plays a major role in deciding or determining who actually develops Kawasaki disease. "And we think this because if Japanese children move to America which has a relatively low rate, their risk remains as high as it would be if they were in Japan. "And the risk of brothers and sisters who have had Kawasaki disease is about 10 times the risk of the general population."So we think that genes are going to be important in determining who actually develops Kawasaki disease when they're exposed to whatever it is that's triggering this illness," he added.Professor Burgner says the findings are an important first step in understanding the disease."Ultimately we'd like to develop a diagnostic test, that's really what the paediatricians are crying out for - a bedside or a diagnostic test for Kawasaki disease, because it's a very difficult diagnosis to make sometimes," he said. "We need better treatment because our best treatment actually fails in 5 to 10 per cent of cases to prevent damage to the heart. "It's not inconceivable. In the future we may be able to develop a vaccine to prevent Kawasaki disease and maybe that will have some impact on future risk of heart attack and things like that.Based on report by David Weber for PM Friday january 9, 2009.
An e-mail I send to Jane Burns M.D.
An e-mail I send to Jane Burns M.D. director of Kawasaki Disease Research Center
Dear Mrs. Gutierrez,thank you for your note. Glad to hear that you are promoting awareness of KD. Attached is our site which you are free to link to your site. Go to the "Finding your Voice" handout and you'll find some ideas about KD activism for parents.We would be grateful if you could let parents know about our genetics study of families with KD children. I am attaching information which you are welcome to post on your site. Parents who are interested should contact me so that we can get mailing addresses and send them a DNA kit. We collect DNA with Scope mouthwash for older children and adults and with blood drawing or mouth swabs for babies.Greg Kurio is a pediatric cardiologist at Oakland Children's Hospital and you might want to link up with him. He is one of my former fellows and very knowledgeable and interested in KD.Re: arthritis. There is no data to suggest that KD kids go one to develop arthritis syndromes any more often that the population at large. Remember that there is a huge ascertainment bias if you collect information from websites since parents with children with problems are more likely to go to websites and post their experience whereas parents whose child had no complications would be less likely. Having said that, we desperately need a national or even just a state-wide registry for children with KD but someone has to come up with the money to do it. We have the expertise to organize this but not the money.Hope this information is helpful. Keep up the good work. We are hoping to have a symposium for parents here in San Diego and have Dr. Kawasaki come over to meet parents the weekend of Nov. 7th so perhaps you could come with a contingent of parents from Northern California.All the best,Jane BurnsJane C. Burns, M.D.Professor and Chief, Division of Allergy, Immunology, and RheumatologyDirector, Kawasaki Disease Research CenterDept. of PediatricsUCSD School of Medicine/ Rady Children's Hospital San Diego9500 Gilman Dr.La Jolla, CA 92093-0641Tel 858-246-0155FAX 858-246-0156________________________________From: VANESSA GUTIERREZ [babybella1983@yahoo.com]Sent: Tuesday, April 07, 2009 11:13 PMTo: Burns, JaneSubject: KD AWARENESSMs. Burns,I am a mother to a KD survivor, my daughter had KD at 4 months old, I am doing my part in helping raise KD awareness here in San Francisco, I have started a website on KD awareness on my space, which has been very helpful to learn more about the aftermath of KD. I have a question : Would you know what are the percentage or chances of a KD child developing arthritis later on in life as a consequence of KD? I have kept in touch with a few other parents of KD children and KD survivors and about 5 of them have told me their children suffer from arthritis, I am worried that my daughter will develop this as she gets older. My pediatrician has helped, yet I am contancting you for I know you are very involved with the KD research and have been for around 30 yrs. Any of your time in reply to my question will be very highly appreciated and I will post it on my blogs as well for other parents to see. I would also like to ask how can I help with promoting KD awareness? I would be interested in participating in any of your researchs that lead to a cause for KD.Thank you for your time,VanessaBelow are the URL to my KD awareness site, my blogger is still under construction, my space page is more developed and informative I have been able to contact several KD families through it."Ask me about KAWASAKI DISEASE!the way we change the world is one person at a time."http://www.myspace.com/kawasakidiseasehttp://www.msplinks.com/MDFodHRwOi8va2F3YXNha2lkaXNlYXNlYXdhcmVuZXNzLmJsb2dzcG90LmNvbS8=
Friday, May 1, 2009
Vasculitis Foundation Awareness Week!
Vasculitis Awareness Week May 3-9, 2009, pls. contact the VF to learn how you can participate!
This is an e-mail Shannon from The Vasculitis Foundation send me, they have a database for children who have had Kawasaki disease, if you would like to have your child added to the database please contact her, send your childs full name and date of birth. They are also having awareness week on May and are asking to please pass out KD cards to aware people of it, if you would like to help out please contact her. ~Vanessa Hello Vanessa,Thank you so much for your email and I have placed Isabella's information in the database. Your url site looks great and we will send you 25 KD disease cards to pass out. They will be better quality if we mail them. Judy Blankenship at jblankenship@vasculitisfoundation.org will mail them. I will let her know.Also, I have forwarded your information to our Executive Director @jakullman@vasculitisfoundation.orgThe packet you will receive is free and I showed you how to download the patient packet. (see below)You will receive (in the packet) a sample newsletter and if you join for $25.00 you will receive the newsletter for one year. The newsletter is very informative and it has patient stories, area contacts and Chapter meeting information, research being performed and the most updated information available in written articles by the medical community.We are so grateful that we heard from you and thank you, again, for ALL you are doing and thanks for making all of the patients with whom you have contact, aware of Awareness Week.Take good care and I have marked you as an Advocate in the database.Sincerely,Shannon
Shannon MorganNew Patient Support and New Chapter CoordinatorVasculitis Foundation Phone: 561-732-6744 (FL)Toll Free: 800-277-9474http://www.msplinks.com/MDFodHRwOi8vd3d3LnZhc2N1bGl0aXNmb3VuZGF0aW9uLm9yZy8=Celebrate Vasculitis Awareness Week May 3-9, 2009. Please contact the VF office to learn how you can participate
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