Kawasaki Disease
Bilitis Rangel
Chamberlain College of Nursing
NR281: Pathophysiology
September 2015

Kawasaki Disease (KD)
Introduction
Many people in the United States are not aware of Kawasaki Disease (KD) as it mainly affects children under the age of five of Asian descent, however in the United States there have been cases in the African American community (Saguil, A.; Fargo, M; and Grogan, S.; 2015). The first case of Kawasaki Disease (KD) presented itself in Japan in 1961, to a 4 year old boy who had the following symptoms high fever, bleeding lips, strawberry tongue and diffuse erythematous of the oral cavity and mucous membrane (Kawasaki, T., Naoe, S. 2014). Kawasaki Disease affects the coronary, small and medium-sized arteries in the body, affecting the blood supply to the muscles in the heart. Kawasaki Disease (KD) is the leading cause of heart disease in children (Laurito, M; Stazi, A; Delogu, A; … and Crea, F; 2014). Kawasaki disease is also known as Kawasaki Syndrome (KS) and mucocutaneous lymph node syndrome as it affects the skin, lymph nodes and mucous membranes in the mouth, nose and throat. The disease starts of with a high fever and a skin rash that results in peeling and while this can be scary to parents, it is treatable and most children recover without any side effects.
Etiology
The etiology of Kawasaki Disease (KD) is unknown, but epidemiological and clinical characteristics show that infectious agents in its pathogenesis are involved. There is a believed hypothesis of a super-antigen which invades through the digestive and respiratory organs which are then processed by the lymphatic system in the organ. The way they have come to this hypothesis is by several findings: skin and mucosal changes, pro-inflammatory cytokines and peripheral t-lymphocytes. And while the investigation for this hypothesis is still on-going, and lack of supporting evidence have yet to support these findings (Takahashi, K., Oharaseki, T., Yokouchi, Y., … Saji, T., 2012).
Pathophysiological Process
Over the years there have been many studies as to the pathologic process of Kawasaki Disease (KD). A recent study conducted on 41 patient’s arterial tissue with Kawasaki Disease (KD) showed that the pathogenesis we thought to be the pathological process of KD actually does not fully explain the complexities of KD arteriopathy. (Rowley, A., 2012). The study showed that the pathologic features in the arteries are best defined as three processes. The first process is self-limited necrotizing arteritis which is inflammation of the blood vessels resulting in neutrophilic necrosis of the arterial walls into the adventitia. The second process is subacute/chronic SA/C vasculitis which is the persistence of inflammation of the arteries which begins at the adventitia and continues to the lumen where plasma cells, lymphocyte and eosinophil infiltration happens and results on the beginning of the third process. The third process is luminal myofibroblastic proliferation (LMP) which is a continuation of SA/C vasculitis which causes the alteration of medial and adventitial smooth muscle cells (SMC) derived myofibroblasts and their matrix products and inflammation which create progressive arterial stenosis or thrombosis, ultimately resulting in ischemia (Takahashi, K., Oharaseki, T., Yokouchi, Y., … Saji, T., 2012).
Clinical Manifestation and Complications Kawasaki Disease (KD) is not particularly easy to identify as it can be misconstrued by other issues or illnesses. But ultimately if a child is irritable, has red around the eyes and dry, red mouth and has a decrease of oral intake and all of this is then followed by an onset fever, more than likely it would be ideal to have the child checked for Kawasaki Disease (KD). The acute stage of KD begins with a fever that reaches between 102-104 degrees Fahrenheit and lasts approximately 7-14 days, conjunctivitis, rash on the body trunk and genitalia; red, dry, cracked lips with a red swollen tongue, usually known as the strawberry tongue. The next stage is called the subacute stage which will result in peeling of the skin, mainly the finger and toe tips, joint pains, vomiting, abdominal pain, and diarrhea. On the third stage which is called convalescent stage the symptoms from the previous two stages slowly start to go away and signs of cardiac issues will begin to show (Saguil, A. ; Fargo, M; and Grogan, S.; 2015).
Diagnostics
There is no specific diagnostic test for Kawasaki Disease (KD). It is a combination of signs and symptoms, followed by laboratory and echocardiographic findings that help diagnose the disease. Blood work showing elevated C-reactive protein levels and erythrocyte sedimentation rate usually help with a diagnosis combined with an electrocardiogram to check for cardiac abnormalities. Aside from these diagnostic tactics, KD is really hard to diagnose and it is usually missed by parents and doctors within the initial onset of the disease as the more relevant symptoms do not show until around the 5th day (Laurito, M., Stazi, A., Delogu, D., … Crea, F. 2014).
Affected Health Patterns with Specific Impact Gordon’s Functional Health Patterns is used to help nurses identify and diagnose potential problems and ultimately help treat patients accurately per their disease. There are a total of eleven functional health patterns. Kawasaki Disease (KD) ultimately impacts 4 of them, Elimination, Cognitive-perceptual, Activity-exercise, and Nutritional-metabolic.
When a child presents with Kawasaki Disease (KD) they have symptoms such as diarrhea which impacts the elimination pattern which is the pattern of excretory function. They also present with symptoms such as swelling of the mouth, skin and mucous membranes which impact the nutritional-metabolic pattern which is the pattern of food and fluid consumption. These symptoms coincide with the pain due to the swelling in the mouth and skin which in result impact the cognitive-perceptual pattern which is the sensory-perceptual and cognitive pattern. The final pattern affected by KD is activity-exercise, which is the recreation/activity pattern, the disease ultimately affects the cardiovascular system which will result in weakness and fatigue and the child will not be able to be as active as it would normally be. (Edelman, C., Kudzman, E., & Mandle, C., 2014). Conclusion Kawasaki Disease (KD) is very serious and all parents should be aware of the symptoms of this disease, particularly because it affects children under the age of five and they may not be able to express the symptoms verbally which will mean the parent needs to be extremely alert. We now know what it is, the signs and symptoms and their developments and how to diagnose. The disease is ultimately treatable and cardiac abnormalities can be avoidable by ensuring to pay attention to the initial signs of the disease.

References
Edelman, C., Kudzman, E., & Mandle, C. ,(2014). Health Promotions and the individual. Health Promotion Throughout the Lifespan (8th ed., pp. 126-127). Boston, Massacussets: Mosby.
Kawasaki, T., Naoe, S. (2014) History of Kawasaki Disease. Clinical and Experimental Nephrology, 18, 301-304. doi: 10.1007/s10157-013-0877-6
Laurito, M., Stazi, A., Delogu, D., Milo, M., Battipaglia, I., Scalone, G., Infusino, F., Villano, A., Russo, G., Iannotta, R., Saracino, A., Parrinello, R., Figliozzi, S., Sestito, A., Romagnoli, C., Lanza, G., and Crea, F. (2014) Endothelial and Platelet Function in Children with Previous Kawasaki Disease. Angiology, 65(8), 716-722
Rowley, A. (2012) Kawasaki Disease – Genetics, Pathology, and a need for earlier diagnosis and treatment. Contemporary Pediatrics, 18-24.
Saguil, A., Fargo, M., Grogan, S. (2015) Diagnosis and Management of Kawasaki Disease. American Family Physician, 91(6), 365-372.
Takahash, K., Oharaseki, T., Yokouch, Y., Naoe, S., Saji, T. (2013) Kawasaki Disease: basic and pathological findings, 17, 690-693. doi: 10.1007/s1015-012-0734-2