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kawasaki disease

Kawasaki disease

Kawasaki disease is a rare condition in children that involves inflammation of the blood vessels.
Causes

Kawasaki disease occurs most frequently in Japan, where the disease was first discovered. In the United States, after congenital heart defects, Kawasaki disease is the leading cause of heart disease in children. Most of these patients are younger than age 5. The disease occurs more often in boys than in girls.

Kawasaki disease is a poorly understood illness. The cause has not been determined. It may be an autoimmune disorder. The disorder affects the mucus membranes, lymph nodes, walls of the blood vessels, and the heart.

Kawasaki disease can cause inflammation of blood vessels in the arteries, especially the coronary arteries. This inflammation can lead to aneurysms. An aneurysm can lead to a heart attack, even in young children, although this is rare.
Symptoms

Kawasaki disease often begins with a high and persistent fever greater than 102 °F, often as high as 104 °F. A persistent fever lasting at least 5 days is considered a classic sign. The fever may last for up to 2 weeks and does not usually go away with normal doses of acetaminophen (Tylenol) or ibuprofen.

Other symptoms often include:
Extremely bloodshot or red eyes (without pus or drainage)
Bright red, chapped, or cracked lips
Red mucous membranes in the mouth
Strawberry tongue, white coating on the tongue, or prominent red bumps on the back of the tongue
Red palms of the hands and the soles of the feet
Swollen hands and feet
Skin rashes on the middle of the body, NOT blister-like
Peeling skin in the genital area, hands, and feet (especially around the nails, palms, and soles)
Swollen lymph nodes (frequently only one lymph node is swollen), particularly in the neck area
Joint pain and swelling, frequently on both sides of the body

Additional symptoms may include:
Irritability
Diarrhea, vomiting, and abdominal pain
Cough and runny nose

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Comment by Pricilla Chelvam on August 31, 2011 at 8:53pm: Abstract:

Despite a high Asian incidence of Kawasaki disease (KD), there is a paucity of reports from India. We report a series of 6 children from Bombay and review published Indian literature (PUBMED search). We highlight certain features and problems of this disease that may be unique to our setting. We describe a new effort in India to educate our medical colleagues about KD and improve diagnosis and treatment.

Introduction:

Although KD has been reported from all over the world, Asia has reported a higher incidence in comparison to other continents. Only a paltry 38 cases from India have been reported in the pediatric literature over the last 25 years. This number of reported cases in one of the most populated countries of the world suggests gross under-diagnosis and under-reporting.

Case reports and review:

Over a period of eight months, between March-October 2002, we diagnosed 6 children with KD using established clinical criteria. [1] All children in our series underwent echocardiography studies at diagnosis and at 6 weeks follow up, and received intravenous immunoglobulin (2gm/kg) with standard aspirin therapy. We reviewed Indian case reports utilizing a PUBMED search through March 2003 and found a total of 14 citations. (Table 1) There were 32 enumerated cases, of which 28 cases had been described in detail. This analysis includes the 28 cases described and our series of 6 patients.

Demographic distribution of our series and collated Indian data is shown in Table 2. More than 70% of the children fell in the typical age category of 1–5 years with the youngest child being 80 days old. A high male to female ratio of 10:1 was noted in the overall data. There were 5 atypical / incomplete cases described. Atypical presentations were seen in children outside the 1 to 5 year age range (4 of 5 patients), and 3 of them demonstrated echocardiographic abnormalities.

The prevalence of the diagnostic clinical features seen is summarized in Table 3. Notably, amongst the diagnostic clinical criteria, lymphadenopathy was seen in 88% in our series. Of the other clinical features described (Table 4), irritability was by far the commonest followed by arthritis and diarrhea in equal measure. One child had reactivation of the BCG vaccination site. Six patients (18%) showed coronary/cardiac anomalies on 2 D echocardiography (Table 5). While 2 patients had developed coronary aneurysms, 3 others demonstrated dilatation of the coronary arteries and one child had mitral regurgitation as an isolated manifestation.

IVIG was not administered to 15 % of cases, and in up to 33% of cases cost constraints led to the use of staggered or incomplete treatment regimes (Table 6). Of the 6 patients with coronary involvement, 5 received IVIG. Three of these children showed complete regression of their cardiac lesions.

Discussion:

In Japan the current reported incidence rate for KD is 120-150 cases per 100,000 children < 5 years. [2] A study conducted in California showed Asians to have the highest incidence as compared to others in the study population. [3] Similarly, a report from Birmingham, UK estimated the annual incidence of KD at 5.5 per 100,000 children < 5 years with the highest incidence being in the Indian subcontinent Asian children (14.6 per 100,000). [4] Despite this, reports from India are scant and the possible reasons for this are discussed below. We suspect that the gender bias in seeking medical attention may be responsible for the high male to female ratio reported from our country.

The incidence of lymphadenopathy seen in our population (88%) is higher than that reported in other studies. [5,6]. We speculate that this may be due to the high background prevalence of recurrent upper respiratory infections and poor dental hygiene in our population. The occurrences of atypical/incomplete KD (15%) and coronary involvement (15%) are slightly lower to those reported elsewhere [5,7]. With just a quarter of the pat