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Kawasaki Disease -

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(generated from captions) MARK: It's an infectious disease that literally breaks the hearts of thousands of children worldwide. But we know surprisingly little about it, not even its cause. What the infection is, to it, don't know. Why some people are more prone

And why people react to it, and have a lot of damage to the coronary arteries, we don't really know. Kawasaki disease is a mystery that has grabbed the imagination of paediatricians all over the world. Medical research races to keep up as it spreads to every continent, and in many countries, it's on the rise. Fascinatingly, it's increasing with rapid industrialisation. So as countries move from a rural to industrial economy, there seems to be an explosion, if you like, of Kawasaki disease. It was first diagnosed in Japan by Dr Tomisaku Kawasaki in 1967. Now 88, Dr Kawasaki still hopes to reveal the secrets of the disease that bears his name. After more than 40 years of research, Kawasaki disease remains a mystery. We just don't know if it's a virus, or bacteria or something else. But we do know it crosses oceans to be the most common cause of childhood heart disease in the developed world, including Australia. It's hard to believe that a few months ago five-year-old Josh was gripped by pain and fever. After a week of getting worse, his mum rushed him to hospital. By then I had to carry him, 'cause he couldn't walk. His eyes were bloodshot and his still had a temperature, it was now 39 point something. They admitted him straightaway. Josh is one of 200 cases of Kawasaki disease recorded in Australia each year, mostly children less than five years old. If undetected in childhood, Kawasaki can lead to heart disease later in life. DAVID: This is more common than meningococcal disease, and children with Kawasaki disease have very high fevers, up to 40 degrees, and the fevers go on for days. So you need to have a fever for four or five days before you can make the diagnosis. They often have a bright red rash, red lips, red eyes, bloodshot eyes, swollen and painful hands and feet, and the older children will have a swollen gland in the neck often. When the visible symptoms have faded away, the real worry is left hidden here, around the heart. Ironically, it's the body's own defence system that does the damage. The disease attacks the coronary arteries - that supply blood to the heart muscle - by triggering an over-reaction of the immune system. Cells of the immune system invade and inflame the artery walls. How to stop the damage? Dampen the down the immune response as quickly as possible. The only proven treatment to Kawasaki disease at the moment is immunoglobulin, which is antibody taken from blood donors. It's the sort of clear part of blood, not the red part of blood. Used within ten days of the infection, the immunoglobulin dramatically reduces the risk of heart damage, and makes the fever disappear. That's exactly what happened. They treated him and within 24 hours he was a different child. His bloodshot eyes had almost gone, and his temperature had come right down to normal. But that's not the end of hospitals for kids sick with Kawasaki disease. The next visit is an echocardiogram. an ultrasound to check for swollen arteries called aneurysms. MICHAEL: The treatment's goal is to protect the heart. We don't want the coronary arteries damaged. We want the heart muscle to be working as well as possible for a long life. In 5% of cases, the treatment fails. This is an X-ray image of an aneurysm in a child, the potentially fatal impact of Kawasaki disease. Unfortunately this one's probably the worst one we've seen here for many years. What you can see here is a very enlarged coronary artery through that section over a long distance. It's a bit like a river. If you've got a river bend and a bit of a bank there, then the water swirls around it. Little clots may form there, and if those little clots then go out into the periphery, and into the smaller blood vessels, it may cause temporary blockage of the blood supply, and cause the heart muscles to suffer because of that. And that can require surgery, something that Cameron Mates and his mother Shirley know all too well. Here you are with Heart Man. Hearty Heart they call him. Back in 1994, Cameron caught Kawasaki disease, and spent his seventh birthday in hospital. Out of that he had this massive aneurysm in his heart. At that point I'd never really heard much. You don't associate heart disease with children, really. What's that big black ball there?

That's the aneurysm that you can see. A typical coronary artery is 3 to 4mm, and mine is 18mm. So it's really swollen up. Yes, it is. 8mm is what they consider as a giant one. What do they call yours? A humungous one? By the time he was 14, young Cameron needed

an adult-sized coronary bypass operation to save his life. I'm particularly interested in why some kids get sick and other don't, when really kids are exposed to the same bugs and triggers all the time. I think there's a lot more out there, probably years and years ago, there's a lot more that's just never been diagnosed. (Heart beats) It looks like whatever causes Kawasaki disease is spread throughout the population. So the question is this - why do so few people catch it when so many are exposed? There's good evidence that genetics plays a major role in deciding or determining who gets Kawasaki disease. For example, we know that Kawasaki disease is 20 times more common in Japanese than it is in Caucasians in Europe and in Australia. But when Japanese families moved to the US, which is a low-incidence country, their children have an incidence that's as high as it is in Japan, in fact, it's slightly higher. Now there's a surprising twist in this story. New research has found not the cause of Kawasaki disease, but perhaps what it's carried by. The wind. There are no examples that I know of in human medicine where there has been trans-oceanic transport of a human pathogen carried by dust particles on the wind. Dr Jane Burns is speaking at the international conference on Kawasaki disease. What she has to say in a lecture called Blowing In The Wind stuns the medical community. This will be a revolutionary new concept in transmission of human disease. Over the last 40 years, when north-westerly winds blow over Japan, there are spikes in Kawasaki cases. That's remarkable in itself. But when the winds from Asia connect with winds crossing from the North Pacific, there are epidemics on both sides of the ocean at the same time. JANE: If it's something that lofts up into these wind currents over the plains of Central Asia, and then can drop out of the sky on children in Japan,

but then can also follow these wind portals across the Pacific Ocean, and then rain down on children here in the United States, particularly here in Southern California, then it must have some kind of particle mass. Atmospheric scientists are flying high-tech sampling instruments above Japan in the hope of isolating what those particles might be. If in fact it's true, that infectious agents can move around the planet on dust particles, that would also open up the possibility that anthrax or influenza or other infectious agents might also move around the planet that way too. This is a detective story with plenty of suspects. Dave Burgner has a hunch of his own. I think more then one bug can act as a trigger, and actually I suspect that there might be two bugs acting at once. Research continues, but the answer to this mystery is elusive. Whatever the cause, getting the correct diagnosis as quickly as possible is crucial. And if you get fobbed off by your doctor or your emergency department, as the fever gets beyond four or five days,

just get them to consider Kawasaki disease. In the shifting winds of a changing world, a child's heart could depend on it.