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  Kawasaki disease

Kawasaki disease or Kawasaki syndrome otherwise known as mucocutaneous lymph node syndrome/disease and infantile polyarteritis, is a self-limiting non-contagious vasculitis which is poorly understood. This condition affects many organs in the body, including the skin, mucous membranes, lymph nodes, blood vessel walls, and the heart. The disease got its name from a Japanese physician who first described the disease in 1967 named Dr. Tomisaku Kawasaki. The highest incidence of Kawasaki disease occurs in Japan, while there is an increasing incidence being the most common cause of acquired heart disease among children in the United States. Kawasaki disease afflicts predominantly young children, in fact, 80% of patients are below 5 years of age. Additional predisposing risk factors include Asian race and the male sex.

Kawasaki disease Skin diseases
Kawasaki disease

The causative agent of Kawasaki disease remains to be unknown. However, an immune- mediated mechanism and an unidentified virus has been presently hypothesized as possible causative factors. Therefore, continuous research is being done to eventually discover a definitive toxin or antigenic substance that is specifically causing the disease.

Symptoms of Kawasaki disease often begin with a high-grade fever (greater than 39 °C or 102 °F; even as high as 40 °C or 104 °F) that persists constantly for up to two weeks duration and unresponsive to normal doses of acetaminophen and ibuprofen. This is accompanied normally by irritability. Children sufferers eventually develop red eyes (conjunctivitis) without pus, known as “conjunctival injection”, red oral mucous membranes, bright red, chapped or cracked lips, a "strawberry tongue" (markedly red bumps called papillae on the back of the tongue) and swollen neck lymph nodes. Skin rashes may appear early in many forms except for blisters on the trunk area. Other associated symptoms include: rapid heart beat (tachycardia), red and swollen hands and feet, bilateral joint pains and swelling. The later phases of the illness present with skin peeling particularly in the genital area, palms, especially around the nails, and soles of the feet. During the course of the illness, some of these symptoms may come and go.

The heart complication is the most important aspect in Kawasaki disease because it can cause coronary (blood vessels supplying the heart muscle) inflammation and subsequent coronary artery aneurysms may develop. If this is left untreated even in young children, significant risk of sudden cardiac death due to myocardial infarction (heart attack) occurs.

The diagnosis of Kawasaki disease is based its clinical signs and symptoms, and there is no specific test that can confirm that an individual has this condition. Therefore, it’s normally quite difficult to establish the diagnosis, especially during the early course of illness. Most of the time, children are not diagnosed until after many visits to several physicians. Differential diagnosis considered may include: scarlet fever, toxic shock syndrome (TSS), and juvenile idiopathic arthritis. There are some laboratory tests that may aid in establishing the diagnosis, these are: 1. complete blood count (showing normocytic anemia and thrombocytosis, in later phase) ; 2. elevated erythrocyte sedimentation rate; 3. elevated C-reactive protein; 4. liver function tests (LFTs) revealing liver inflammation and low albumin. Other adjunct tests which may or may not be requested include: a. ECG b. 2D-ECHO c. USG or CT scan showing enlarged gallbladder d. urinalysis showing pyuria and proteinuria without bacteria e. spinal tap f. angiography , the gold standard to detect coronary aneurysms.

Children with Kawasaki disease should be hospitalized and managed by an experienced pediatric cardiologist and pediatric infectious disease specialists despite the fact that no infectious agent has been demonstrated. Treatment should be started immediately once the diagnosis has been established to greatly reduce the risk of coronary artery aneurysms. Rapid recovery may be expected from acute symptoms once treated early. In cases of relapse, which is also common re-hospitalization and re-treatment is warranted.

The standard treatment for Kawasaki syndrome is high dose Intravenous gamma globulin (IVIG). Marked improvement of symptoms is usually noted within 24 hours of drug administration. Although not as effective as IVIG alone, salicylate therapy, particularly aspirin, remains a vital aspect in the treatment. Aspirin is started at high doses until the fever subsides, and then intake is continued at a lower dose but it is not normally prescribed for children (except for Kawasaki disease and a few other indications) due to its association with Reye's syndrome. When the standard pharmacologic treatment fails to resolve the symptoms, corticosteroids have also been used but have not been considered as first-line of drug choice for Kawasaki therapy.

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