Kawasaki Disease Key Terms

A list of medical terminology associated with Kawasaki disease with definitions in plain English and links to more detailed information.

Symptoms:

Inflammation – inflammation is the protective result of some kind of cellular damage in the body and is marked by redness, swelling, and pain.  When this inflammation occurs in the blood vessels, it is called vasculitis.  In Kawasaki disease, the small to medium blood vessels through the body may experience inflammation, particularly in the coronary arteries of the heart.  Inflammation can also be seen by the chapped lips, strawberry tongue, rash, and reddened palms and soles.

Conjunctiva – the conjunctiva is a thin membrane that covers the inner surface of the eyelid and the white part of the eyeball. 

Nonexudative Bilateral Conjunctivitis – this is a fancy way of saying the eyes are red.  Conjunctivitis is inflammation of the conjunctiva; nonexudative means there is no puss or fluid; and bilateral means the redness occurs in both eyes. 

Anterior Uveitis – anterior uveitis is inflammation of the middle layer of the eye.

Oropharyngeal changes - oropharyngeal refers to the changes in the lips, mouth, and tongue.  In KD this includes chapped, cracked lips and strawberry tongue.

Strawberry Tongue – with strawberry tongue, inflammation can cause the tongue to become a deep red color and have small, raised bumps – eerily resembling a strawberry. 

Lip Fissures – lip fissures refer to cracked lips.

Lymphadenopathy – lymphadenopathy is the swelling of a lymph node.  During KD, lymphadenopathy occurs most frequently in one of the lymph nodes of the neck. 

Polymorphous Rash – a rash often accompanies Kawasaki disease, most often found on the trunk of the body.  Polymorphous refers to the fact that the rash can look different on different people. 

Exanthem – exanthema is a skin disruption (fancy word for rash) that is usually accompanied by fever, like in Kawasaki disease. 

Erythema – erythema is a fancy word for redness. 

Edema – edema is a fancy word for swelling caused by excess fluid.  In KD, erythema and edema on the hands and feet are a classic symptom. 

Desquamation – desquamation is a fancy word for the peeling or shedding of the skin.  In the later stages of Kawasaki disease, peeling of the skin on the hands and feet may occur. 

Beau’s Lines – Beau’s lines are horizontal depressions or grooves in the surface of the nails.  Beau’s lines can occur 1-2 months after the initial fever of Kawasaki disease.

Perianal Erythema – perianal erythema refers to redness around the anus.

Laboratory Terminology:

Urinalysis – a urinalysis is a test that evaluates a sample of urine.  In KD, a urinalysis may show sterile pyuria, pus, or protein in the urine. 

Sterile Pyuria – sterile pyuria is the presence of elevated white blood cells found during a urinalysis.

Acute-Phase Reactants – acute-phase reactants are substances in the blood (proteins) that will increase when something is wrong in the body – like during an infection, injury, or trauma.  In Kawasaki disease, acute-phase reactants are almost universally elevated at first and usually return to normal 6-10 weeks after the onset of the illness.  These reactants may be measured with various blood tests, including ESR and CRP.

ESR/SED – ESR stands for Erythrocyte Sedimentation Rate and can also be referred to as SED rate.  This is a blood test that will indirectly measure the amount of inflammation in the body – it will measure the acute-phase reactants, which means that the SED rate will usually be elevated during the beginning phases of Kawasaki disease.

CRP – CRP stands for C-reactive Protein Levels.  This is another blood test that will show if there is inflammation in the body and may be elevated during KD. 

Alpha1-Antitrypsin – Alphal-Antitypsin (AAT) is a protein – an acute-phase reactant – found in the blood that may increase during the inflammation found in Kawasaki disease. 

Meprin A and Filamin C – These are 2 urine proteins that researchers have linked to KD as possible biomarkers of Kawasaki disease.

CBC – CBC stands for Complete Blood Count.  This blood test will measure a great deal of things including:

Red blood cells, which carry oxygen

White blood cells, which fight infection

Hemoglobin, the oxygen-carrying protein in red blood cells

Hematocrit, the proportion of red blood cells to the fluid component, or plasma, in your blood

Platelets, which help with blood clotting

During the acute phase of Kawasaki disease, a CBC may show an elevated white blood count (WBC), elevated platelet count, and/or normochromic anemia.

Normochromic Anemia – Anemia is a lack of the proper amount of red blood cells.  There are many different kinds of anemia; in Kawasaki disease, normochromic anemia may be found.  This type of anemia occurs when the hemoglobin content of the red cells as measured by the MCHC is in the normal range.

ECG – an ECG (or EKG) is short for Electrocardiogram. This test uses electrodes attached to the skin to measure the electrical impulses of your child's heartbeat.  It is noninvasive and painless, although you will have to remove the sticky circles from the skin after the test.  

Echo – an echo refers to an Echocardiogram. This test uses ultrasound images to show how well the heart is functioning and provides indirect evidence on how the coronary arteries are functioning.  This is one of the most important tests performed during a Kawasaki disease diagnosis. 

Magnetic Resonance Imaging (MRI) – an MRI creates an image of the body or part of the body using powerful magnets and radio waves.  It can be used to get a clearer image of coronary artery damage associated with Kawasaki disease.

Computed Tomography (CT) – a CT or CAT scan is a special x-ray that takes many cross-section images of the body or part of the body.  It can be used to get a clearer image of coronary artery damage associated with Kawasaki disease.

Cardiac Angiography – cardiac angiography is a procedure that guides a special dye, aided by x-ray, through the arteries of the heart.  It is often done in conjunction with cardiac catheterization. 

Cardiac Catheterization – cardiac catheterization involves the insertion of a small tube – a catheter – into one side of the heart.  Many diagnostic tests can be performed during a cardiac catheterization.  With Kawasaki disease, angiography and catheterization are only performed when there are complications such as coronary artery aneurysms. 

Heart Complications:

Coronary Artery – Coronary arteries run along the outside of the heart and are responsible for providing oxygen-rich blood to the heart muscle.  Coronary artery aneurysms are the most common serious complication involved with Kawasaki disease. 

Aneurysm – An aneurysm is a weakened area in the wall of a blood vessel which causes an outpouching or bulge. Aneurysms may be fatal if these weak areas burst, resulting in uncontrollable bleeding.

Arrhythmia – An arrhythmia is any deviation from a normal heart beat.

Arteritis – arteritis is inflammation of an artery.

Thrombocytosis – thrombocytosis is a condition where your body produces too many platelets.

Cardiomegaly – cardiomegaly is an enlarged heart.

Myocarditis – myocarditis is inflammation of the heart muscle (myocardium).

Pericarditis – pericarditis causes swelling and irritation of the pericardium – a thin membrane that surrounds your heart. 

Myocardial Infarction – a myocardial infarction is a heart attack. 

For more information on Kawasaki disease, check out the Kawasaki Disease Resource List. 

Kawasaki Disease and Cholesterol

I very often get asked specific questions about Kawasaki Disease and __________. While I feel that my DSK Kawasaki Disease Resource List is very useful, it can sometimes get overwhelming to try and find a lot of information on one very narrow subject.  So I'd like to start having in depth posts that give as much information as I can wrestle up on a subject and give everyone a chance to weigh in with their opinions. Let's see how it goes!

I was recently asked if there are specific statistics on how many KD kids are diagnosed with high cholesterol. The short answer is... yes. The long answer is... it's way more complicated than a single statistic. So complicated that I didn't feel like I covered my bases until I had 20 links to share with you. And this is just breaking the tip of the iceberg.

I've arranged the sources in chronological order; I feel it is a fair way to organize them and I like the timeline feel. I'm not giving an opinion of what I think about the information. I've tried to give a balanced amount of information and include different viewpoints that are available. You will notice that many of these articles may sometimes contradict each other. I don't like to try and sway anybody. I'm seeking answers just like you are. And I always like to hear as many sides as I can! I also assume that you all feel the same way.

Many of these links are specifically about KD and cholesterol, many are about kids and cholesterol, and many are just about cholesterol. The last two are about the very latest findings in regards to KD and heart disease in general.  I find each one gives me a better understanding of how to proceed with my KD kid. OK, here we go.

1.  "There was no correlation between the serum cholesterol concentrations and coronary artery abnormalities. These data lead us to infer that KD does not cause such permanent changes in cholesterol metabolism as to be considered a risk factor for atherosclerosis beyond that caused by the disease itself.” 1991, Source: http://www.ncbi.nlm.nih.gov/pubmed/1919886

2.  "We found that Kawasaki syndrome is associated with significant abnormalities in lipid profile. In the earliest days of illness, mean plasma concentrations of total cholesterol and HDL cholesterol were profoundly depressed, whereas mean triglyceride concentration was very high. Total cholesterol values rapidly returned to normal and remained stable more than 3 months after the onset of illness. HDL cholesterol concentration recovered more slowly after illness onset, and mean HDL cholesterol concentration was significantly lower than expected, even in the period, more than 3 years after illness onset. The lipid findings early in the course of Kawasaki syndrome are consistent with those delineated in a variety of acute infections. Both acute bacterial and viral infections are associated with diminished concentrations of low density lipoprotein and HDL cholesterol and with increased concentrations of very low density lipoprotein cholesterol, at least in part attributable to concurrent reductions of lipoprotein lipase and hepatic lipase activities. Altered lipid profile has been demonstrated to be related to the actions of a variety of structurally distinct cytokines (e.g., tumor necrosis factors, interleukins, interferony) released during the host response to infection. The changes in lipoprotein metabolism after acute infections are temporary, although recovery of lipid profile has been reported to be delayed as long as 1 month after disease onset in 80% of patients.23 The persistence of low HDL cholesterol for many years in our sample suggests a more lasting effect of Kawasaki syndrome on endothelial function, perhaps attributable to diminished activity of lipoprotein lipase. This enzyme resides on the capillary walls of most tissues and functions at the luminal surface of the vascular endothelium.” 1991, Source: http://circ.ahajournals.org/content/84/2/625.long

3.  "Lipoproteins participate in the acute response to inflammation. In animal models of acute inflammation and in adult humans with acute myocardial infarction, high density lipoprotein (HDL) levels decrease and serum amyloid A(SAA) increases and is incorporated into HDL. We previously showed that HDL cholesterol and apolipoprotein A1 (apoA1) levels are dramatically low in acute Kawasaki disease (KD) and that the decline is associated with increased SAA. In this study we prospectively assessed the time course of changes in HDL composition in children with acute KD. 12 KD children had plasma obtained before initiation of treatment; 7 had follow-up specimens obtained 2 weeks(n=6) or 6 weeks later. Total cholesterol, triglycerides (TG), HDL cholesterol total and SAA in HDL, core TG in HDL (as%), and apoA1 were measured in each specimen. SAA increased and was present in HDL acutely but not at follow-up. At diagnosis, mean total cholesterol (133 mg/dl), HDL cholesterol (20 mg/dl), and apoA1 (52 mg/dl) levels were below the normal range while core TG was increased (52%). In paired comparisons, total cholesterol, HDL cholesterol and apoA1 all increased (+29 mg/dl, p<0.05; +14 mg/dl, p< 0.0001; +48 mg/dl, p<0.0001 respectively), whereas core TG decreased by 12% (p<0.05) despite no change in plasma TG levels. These studies describe participation of HDL in the acute phase response of KD; this response includes incorporation of SAA into HDL and the recruitment of core TG in exchange for cholesterol ester in the particle. Recovery towards normal begins within 2 weeks of therapy. This is the first demonstration of the participation of HDL in the acute phase response in children.” 1996, Source: http://www.nature.com/pr/journal/v39/n4s/full/pr1996291a.html

4.  "In several reports, abnormal convalescent serum lipid profiles have been identified after the acute febrile phase of Kawasaki disease in a subset of patients. In most reports these profiles returned toward normal by 1 year after onset of the disease. Additional studies to confirm these observations are needed; for now, counseling of parents and patients should conform with standard recommendations on nutrition for children and adolescents.  For patients whose lipid profiles are abnormal, the physician may remeasure lipid levels a year later.”  1998, Source: http://www.besancon-cardio.org/recommandations/kawas-aha.htm

5.  "Subjects with low TC levels (<189 mg/dL) are at higher risk of dying even when many related factors have been taken into account. Although more data are needed to clarify the association between TC and all-cause mortality in older individuals, physicians may want to regard very low levels of cholesterol as potential warning signs of occult disease or as signals of rapidly declining health.”  2003, Source: http://www.ncbi.nlm.nih.gov/pubmed/12834520

6.  "This study demonstrates that children with a history of KD have an adverse cardiovascular risk profile long after resolution of the acute inflammatory illness. In those with coronary aneurysms, the profile is characterized by low HDL cholesterol and apoA-I levels, high apoB levels, and increased peripheral conduit arterial stiffness. In those without coronary complications, apoB levels and brachioradial arterial stiffness are likewise increased. Although the LDL cholesterol levels are higher in patients than in controls, the difference did not reach statistical significance. In addition, we did not find any significant differences in total plasma homocysteine levels and systemic BP between patients and controls.  
     Lipid abnormalities in the acute phase of KD, with decreased total cholesterol, HDL cholesterol, and apoA-I levels, are reminiscent of those in acute infection and inflammation. Although several studies have suggested that such changes are transient, Newburger et al. have reported that HDL cholesterol levels remain low as long as three years after the initial illness. The findings of the present study, performed at a mean of 7.1 years after the initial illness, agree with those of Newburger et al. and further clarify that low HDL cholesterol levels are confined to patients with coronary aneurysm formation. As the severity of vasculitis in the acute phase is, to some extent, reflected by development of coronary lesions, our findings suggest that the degree of inflammation in the acute phase may have important bearings on late lipid abnormalities.
     The changes in cholesterol and lipoprotein levels long term after KD mimic those proposed to be atherogenic. Nonetheless, the underlying mechanisms remain speculative. Endothelial dysfunction has been shown years after resolution of the acute illness. Diminished lipoprotein lipase activity with reduced generation of HDL cholesterol, as a result of endothelial dysfunction, has been proposed as a possible mechanism. Furthermore, inhibition of lipoprotein lipase may decrease apoA-I levels by increasing its catabolism. The cause for the higher apoB and LDL cholesterol levels is unknown. Nonetheless, increased LDL cholesterol levels have been described in adults with chronic inflammation due to rheumatoid arthritis. Indeed, there is increasing evidence that low-grade vasculitis continues unabated after the end of the acute phase of KD. The changes in the lipid profile in our cohort may hence be a reflection of such a continued low-grade inflammatory process.” 2004, Source: http://content.onlinejacc.org/article.aspx?articleid=1132896#bib29

7.  "All children who have had Kawasaki disease should adopt a heart-healthy lifestyle (that is, no smoking, regular exercise, heart-healthy diet) and, if needed, be treated for risk factors for adult-type coronary artery disease, such as high blood pressure and high cholesterol. Children in whom coronary enlargement was never detected return for follow-up infrequently.” 2008, Source: http://circ.ahajournals.org/content/118/7/e110.full

8.  "A meta-analysis of prospective epidemiologic studies showed that there is no significant evidence for concluding that dietary saturated fat is associated with an increased risk of CHD or CVD. More data are needed to elucidate whether CVD risks are likely to be influenced by the specific nutrients used to replace saturated fat.” 2010, Source: http://ajcn.nutrition.org/content/91/3/535.long

9.  Patients who form coronary artery aneurysms of any size in the acute phase are definitely at higher risk for coronary artery disease in early adulthood and later in life. Smaller aneurysms do usually regress and appear normal after the acute phase, but risk of coronary artery occlusion in later life is still increased. Larger (greater than 8 millimeters in diameter) aneurysms remain a lifelong problem affecting normal blood flow and causing blood clots. They can also cause cholesterol plaques to form that lead to blockages. 2011, Source: http://www.cnn.com/2011/HEALTH/expert.q.a/10/05/kawasaki.syndrome.brawley/

10.  "A significant change from previous guidelines is the new recommendation that all children be screened for high cholesterol at least once between the ages of 9 and 11 years, and again between ages 17 and 21 years. The NCEP Expert Panel on Blood Cholesterol Levels in Children and Adolescents, issued in 1992, instead called for screening only children with a family history of heart disease or high cholesterol. Physicians now will be able to use a non-HDL cholesterol test that does not require children to fast; children with abnormal results should be followed up with a fasting lipid profile.”  2011, Source: http://www.aap.org/en-us/about-the-aap/aap-press-room/pages/Physicians-Recommend-all-Children,-Ages-9-11,-Be-Screened-for-Cholesterol.aspx#sthash.Bji3buo0.dpuf

11.  Expert Panel on Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents: Summary Report. 2011, Source: http://pediatrics.aappublications.org/site/misc/2009-2107.pdf

12.  New Guidelines for Universal Child Cholesterol Screening. 2011, Source: http://www.childrensnational.org/files/PDF/DepartmentsAndPrograms/HLK/Cardiology/preventive-cardiology/New-Guidelines-for-Cholesterol-Screening.pdf

13.  "Arditi said the study's findings also may have implications for children with Kawasaki Disease in that they may need to be closely monitored for future development of early-onset atherosclerosis. Also, doctors treating children who have had Kawasaki Disease should closely monitor other known cardiovascular disease risk factors such as obesity, high blood pressure, high cholesterol and smoking, Arditi said.
     Atherosclerosis occurs when fat, cholesterol, and other substances build up in the walls of arteries and form hard structures called plaques. Over the course of years, plaque buildup makes it harder for blood to flow because the plaque narrows arteries and makes them stiffer. When pieces of plaque break off and move to smaller vessels, they can cause stroke, heart attack or pulmonary embolism.
     In the study, which was funded with a grant from the National Institute of Allergy and Infectious Diseases, mice with Kawasaki Disease were fed a high-fat diet and then compared to mice that did not have Kawasaki Disease but did eat the same high-fat diet. The Kawasaki mice developed significantly more atherosclerotic plaque at a younger age.” 2012, Source: http://www.sciencedaily.com/releases/2012/07/120717182957.htm

14.  "Cholesterol is a very misunderstood molecule.  And though many people might not be aware of it, there has long been a vocal minority of doctors, researchers and health professionals who believe that cholesterol and fat have been wrongly convicted as the primary promoters of heart disease. Along with many of our fellow health professionals, we believe that this emphasis on cholesterol has caused us to take our attention off what we believe to be the true promoters of heart diseases – inflammation, oxidative damage, stress and sugar.” 2012, Source: http://www.doctoroz.com/videos/cholesterol-facts-vs-myths

15.  Our finding that 5% of all young adults being evaluated for ischemia may have KD as a cause of their symptoms has important implications for adult cardiologists. The pathology of coronary lesions in patients with a history of KD is very different from the pathology of typical coronary atherosclerosis, and so optimal treatment of each is distinct.19 While typical atherosclerosis is characterized by lipid-laden macrophages, extracellular lipid droplets, and cholesterol crystals, these features are absent in coronary lesions after KD.20 Acutely, KD vasculopathy begins with endothelial cell swelling and subendothelial edema, followed by an intense inflammatory process leading to regions of myointimal proliferation with focal destruction of the internal elastic lamina, medial smooth muscle cell necrosis, and aneurysm formation,21, 22 Over time, myointimal proliferation leads to fibrous scar formation, often with calcification.” 2012, Source: http://circ.ahajournals.org/content/early/2012/05/15/CIRCULATIONAHA.111.082107.full.pdf+html

16.  "Several studies have recently found greater long-term incidence of traditional cardiovascular risk factors in KD patients. Newburger et al first demonstrated that KD patients, particularly those with persistent coronary abnormalities, have decreased high-density lipoprotein cholesterol levels long after acute illness. Cheung et al, in their follow-up of KD patients at a mean of 7 years after illness, found that patients with CAA had not only reduced high-density lipoprotein cholesterol, but also low apolipoprotein A and elevated apolipoprotein B, all risk factors for cardiovascular disease; these patients also demonstrated increased peripheral conduit arterial stiffness, indicating that they have suboptimal cardiovascular health. In comparison, KD patients without CAA demonstrated slightly improved lipid profiles, but were still found to have elevated apolipoprotein B, as well as increased brachio-radial arterial stiffness.” 2013, Source: http://www.pediatr-neonatol.com/article/S1875-9572(12)00222-7/fulltext

17.  "Professor Jeremy Pearson, associate medical director at the British Heart Foundation - which co-funded the study, said: "This analysis of existing data suggests there isn't enough evidence to say that a diet rich in polyunsaturated fats but low in saturated fats reduces the risk of cardiovascular disease. But large scale clinical studies are needed, as these researchers recommend, before making a conclusive judgement.
     "Alongside taking any necessary medication, the best way to stay heart healthy is to stop smoking, stay active, and ensure our whole diet is healthy - and this means considering not only the fats in our diet but also our intake of salt, sugar and fruit and vegetables.” 2014, Source: http://www.huffingtonpost.co.uk/2014/03/18/saturated-fat-not-cause-heart-disease_n_4984392.html

18.  "Current evidence does not clearly support cardiovascular guidelines that encourage high consumption of polyunsaturated fatty acids and low consumption of total saturated fats.” 2014, Source: http://annals.org/article.aspx?articleid=1846638

19.  "This is the largest US study of longer-term cardiac outcomes after KD and reveals a low rate of adverse cardiovascular events through age 21. Additional validation studies, including studies with longer-term follow-up, should be performed.”  2014, Source: http://pediatrics.aappublications.org/content/133/2/e305.abstract

20.  "Study Rundown: Kawasaki Disease (KD) affects medium-sized blood vessels, is most often seen in children younger than 5 years of age, and is sometimes confused for an infectious process given its common presentation of fever. Accurate diagnosis of KD is crucial, as treatment with intravenous immunoglobulin (IVIG) and aspirin has been shown to reduce the risk of coronary aneurysm development secondary to KD’s inflammatory process. This retrospective study compared patient records from children with and without KD, and examined for differences in cardiovascular events. There was no significant difference in cardiovascular events between the two groups. Despite an overall low rate of persistent aneurysms in the KD group, cases of aneurysm development were more likely associated with an adverse event. Retrospective data was obtained from the Kaiser Permanente database, potentially underestimating adverse event data if patients obtained follow-up care elsewhere. This large study reassures the low risk of cardiovascular events in children treated for KD, though even longer-term follow-up is prudent.” 2014, Source: http://www.2minutemedicine.com/cardiovascular-events-in-kawasaki-disease-not-significantly-elevated-over-controls/ 

What are your thoughts on Kawasaki Disease and cholesterol? Comment here or come on over to DSK on Facebook and join the discussion.  Would you like to see more of these in depth articles? If so, please let me know and comment on what topics you'd like to see covered.  

26 Days of Kawasaki Disease Awareness: Day 26

It's finally here! January 26, 2014 - the 4th annual Kawasaki Disease Awareness Day.  Thanks to everyone who joined me in this 26 day endeavor and to everyone who spreads KD awareness in their own way.  During the last month, I have been posting ideas that can raise awareness for KD.  

Here are the past days:

Day 1: Help make 'National Kawasaki Disease Awareness Day' officially recognized at the local, state, and national levels.
Day 2: Create an email signature to promote Kawasaki Disease awareness.
Day 3: Organize an art contest.
Day 4: Pass out KD awareness posters.

Day 5: Write about your Kawasaki Disease experience.

Day 6: Contact news outlets about running a KD story.
Day 7: Decorate your home and car windows with KD awareness.
Day 8: Participate in a KD Clinical Study
Day 9: Share Kawasaki Disease themed infographics and memes.
Day 10: Organize a 5K run for Kawasaki Disease.
Day 11: Get your community involved in KD awareness.
Day 12: Hand out heart lollipops with KD information.
Day 13: Start or join a Kawasaki Disease support group.
Day 14: Buy, make, and wear KD awareness.
Day 15: Organize a charity golf or mini-golf tournament for KD.
Day 16: Organize a local blood drive for KD and donate blood.
Day 17: Get your child's school involved.
Day 18: Make a KD inspired float for a local parade.
Day 19: Organize a charity sporting event for KD.

Day 20: Donate $26 on January 26th and urge others to do the same.
Day 21: Get your public library involved in KD awareness.
Day 22: Get your religious and non-religions organizations  and clubs involved.
Day 23: Attend a Kawasaki Disease Parent Symposium.
Day 24: Help a Kawasaki Disease Survivor with their very own awareness campaign or fundraiser.
Day 25: Make a Kawasaki Disease awareness inspired wreath.

So let's get to the final idea of "26 days of Kawasaki Disease Awareness."

Day 26: Share Kawasaki Disease Facts.

If we are going to spread KD information, we need to strive to spread current, factual information.  Many of the following KD facts are more well known, others may not be so wide spread.  It is crucial to remember that many of these facts come from a variety of different sources and all will be linked to where I originally found the information.  All facts should be considered based on the context of the article that contains it.  

26 Kawasaki Disease Facts, in no particular order:


#1) Kawasaki Disease is the #1 cause of acquired heart disease in children, worldwide. 

#2) Although many theories exist, it has not yet been proven what exactly causes Kawasaki Disease.

#3) 75% of people who are diagnosed with KD are under 5 years of age.

#4) There is no diagnostic test for Kawasaki Disease; it is diagnosed by ruling out other illnesses through diagnostic tests and a thorough history and physical.  

#5) To be diagnosed with KD, you have had a fever for at least five days and show at least 4 out of these 5 symptoms: rash; swollen lymph nodes in the neck; swollen, chapped lips and a strawberry tongue; swollen hands and feet with red palms and/or soles; and bloodshot eyes.

#6) Approximately 25% of KD cases will present as Incomplete Kawasaki Disease, where less than four of the principal features are present but laboratory results or echocardiography suggests the diagnosis of KD.

#7) Number of children <5 diagnosed with KD each year, broken down by country (and rounded to the nearest whole number):

1. Japan - 220 per 100,000
2. Korea - 113 per 100,000
3. Taiwan - 69 per 100,000
4. Canada - 26 per 100,000
5. United States - 19 per 100,000
6. Australia - 10 per 100,000
7. England - 8 per 100,000

#8) If left untreated, Kawasaki Disease will lead to coronary artery aneurysms in 25% of cases.  

#9) Children with Kawasaki Disease have a higher risk of developing atopic dermatitis (eczema) than healthy controls.

#10) 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.

#11) Dr. Tomisaku Kawasaki first saw the illness known later as Kawasaki Disease in 1961.  He wrote his first Japanese-language report on KD on 1967 and his first English-language report in 1974.

#12) The first cases of Kawasaki Disease to be recognized in the United States were in Hawaii in the early 1970s.

#13) Most coronary artery aneurysms resulting from Kawasaki Disease are small to medium in size and 50-67% of small-medium CAA's will regress back to normal size within 1-2 years.

#14) Several lines of evidence suggest that regressed CAA may be at risk for some long-term complications, particularly accelerated atherosclerotic disease.

#15) The American Heart Association and the Japanese Ministry of Health both determine what kind of follow up is needed based on the health of the coronary arteries during and after KD and assign different risk levels. 

#16) Risk Levels:

1. Risk Level I: No coronary artery changes at any stage of illness.
2. Risk Level II: Transient coronary artery dilation which disappears within the first 6-8 weeks.
3. Risk Level III: 1 small-medium coronary artery aneurysm/major coronary artery
4. Risk Level IV: ≥1 large or giant coronary artery aneurysm, or multiple or complex aneurysms in same coronary artery, without obstruction
5. Risk Level V: coronary artery obstruction

#17) Kawasaki Disease has a male to female ratio of 1.5:1.

#18) Rates of Recurrent Kawasaki Disease vary between 0.8% in the United States to 3% in Japan. 

#19) The proportion of patients suffering a recurrence increase with age, while the majority of recurrence occurs within 2 years of the initial attack. 

#20) In rare cases (0.2%), patient can suffer multiple recurrences.

#21) Children who are febrile more than 36 hours after the completion of their initial IVIG infusion require additional therapy.

#22) World's Top Institutions for Kawasaki Disease, according to Expertscape:

• University of California, San Diego
• Hospital for Sick Children, Toronto
• Northwestern University, Chicago
• Chang Gung University, Taiwan
• Boston Children's Hospital
• Academic Medical Center Amsterdam, Netherlands
• Nippon Medical School, Japan

#23) World's Top Specialists in Kawasaki Disease, according to Expertscape:

• Dr. Jane Burns, UCSD
• Dr. Jane Newburger, Boston Children's
• Dr. Standford Shulman, Northwestern
• Dr. Anne Rowley, Northwestern
• Dr. Masato Takahashi, USC
• Dr. Brian McCrindle, Sick Kids
• Dr. Rae Yeung, Sick Kids

#24) Up to 33% of KD patients have at least one concurrent infection at the time of diagnosis, but no correlation between a specific agent and the severity of the disease course has been identified.

#25) There are 4 stages or phases of Kawasaki Disease:

1. Acute
2. Sub-acute
3. Convalescent
4. Chronic

#26) A new study just out is reporting that when the initial outcome of KD is good (no persistent aneurysms), rates of cardiovascular events were no higher than in the control group.  Doctors, of course, want more follow-up studies done to verify these results, but this is VERY good news for lots of little hearts out there! It also illustrates the absolute importance of a prompt diagnosis (within 10 days of fever onset) and the immediate need for a diagnostic test.  

So there you go, 26 facts about Kawasaki Disease that can educate those all over the world and even introduce KD to many who have never heard of it.  

I'd like to say a special thanks to everyone who supported this project.  I'm amazed at the amount of KD awareness I saw spreading all over the world this year, and it gives me great hope for the future.  One day soon, we will have a diagnostic test for KD.  One day soon, every parent will know about this mysterious illness.  One day soon, children's hearts will finally be safe from this silent monster.  One day soon...

Twice!