The Kawasaki disease Is a multisystemic vasculitis (Delgado Rubio, 2016), at a specific level, is a pediatric pathology that causes a Inflammation of the arterial walls of the organism (Mayo Clinic, 2014).
At the clinical level, this disease can affect the arteries, lymph nodes, mucous membranes and even the nervous system (Mayo Clinic, 2014). So, Some of the most common signs and symptoms usually include: fever, conjunctival injection, oral anomalies, cutaneous conditions, cervical lymphadenopathy, Among others (García Rodríguez et al., 2016).
The origin of Kawasaki disease is not yet known with exactness, however, several investigations are underway, relating its etiological cause with Immunological and infectious factors (Arias Cabello, Fernández Álvarez and Ordaz Favila, 2016).
On the other hand, although early identification is complex, confirmation of this disorder is usually performed based on clinical criteria To the clinical and temporal course. In addition, some complementary tests are Blood analysis , he Study of cerebrospinal fluid or he echocardiogram (Bou, 2014).
In terms of treatment, the therapeutic approaches are directed both to the treatment of ongoing pathologies, and to the control and minimization of Medical sequelae Thus, some of the methods employed include the administration of gammaglobulin, acetylsalicylic acid, corticoid or antibodies Monoclonal (Bou, 2014).
Characteristics of Kawasaki disease
Kawasaki disease is a type of Vasculitis , Is also a rare or rare disease that affects mainly the pediatric population and, In particular, to children under the age of 5 (The Royal Child's Hospital Melbourne, 2016).
Vasculitis is a type of disorder that involves a pathological inflammation of the blood vessels of the body, altering the efficient functioning Capillaries, veins, or arteries (National Institutes of Health, 2016).
The different structures of our circulatory system are responsible for transporting and delivering the blood and, therefore, nutrients and oxygen to The whole organism, fundamental for survival.
Thus, when factors related to infectious processes, abnormalities of the immune system or other medical pathologies result in Development of an inflammatory process in the blood vessels, these can be altered in different ways (National Institutes of Health, 2016):
- Partial or complete closure of the blood conduit that hinders the passage of blood to one or more body regions.
- Distension or weakening of the walls of the blood conduit, facilitating the development of aneurysms or malformations.
In this way, when the blood flow fails to reach all organs normally, a wide variety of medical pathologies will occur, Will vary depending on the affected areas.
Some of the medical complications can include heart alterations, fever, aneurysm blood spills, ictus , among others.
Specifically, Kawasaki disease was first described at the clinical level in 1961. Dr. Tomisaku Kawasaki referred to Case of a child who did not exceed 5 years of age and presented a symptom group composed by pathological increase of body temperature, Cervical lymphadenopathy , Skin eruptions, oral anomalies, anemia, etc., all of which are compatible with vasculitis (Prego Petit, 2003).
However, this pathology was not explicitly defined until 1967. Kawasaki, in his clinical report, described about 50 cases of children with a Clinical course characterized by febrile episodes, lymphadenopathy, mucocutaneous alteration and finger flaking (Bou, 2014).
Although numerous cases have been registered in the medical literature, it has not yet been possible to establish the etiological causes that give rise to this pathology. However, its course and clinical judgment has been specified by many authors and medical institutions.
About us
Kawasaki disease is considered one of the most frequent types of vasculitis in the pediatric age (García Rodríguez et al., 2016). Thus, in more 85% of those affected are less than 5 years of age (Bou, 2014).
However, it is a pathology that can occur at any age, although it is infrequent during the neonatal phase (before three months) or in the Teen stage (Prego Petit, 2003).
In addition, in Kawasaki disease a clear pattern of geographical distribution can be observed since it is a pathology that affects Asian population (Bou, 2014).
In a recent national survey in the Japanese region, there was an incidence of approximately 26.9 cases per 100,000 population in age Children, especially between 0 and 4 years of age (Arias Cabello, Fernández Álvarez, Ordaz Favila, 2016).
Apart from this, the most recent epidemiological investigations show that, at present, Kawasaki disease presents a distribution More universal, affected by any ethnic or racial group (Bou, 2014).
In addition, from the first descriptions of this pathology to the year 2015, more than 900 clinical reports related to cases have been published Diagnosed with Kawasaki disease (Sotelo-Cruz, 2016).
On the other hand, in relation to the distribution by sex, it has been observed that it is a predominant pathology in males, with a ratio of 1.5 / 2.1: 1, Against the female sex (Bou, 2014).
In the United States, Kawasaki disease is considered the most frequent type of Heart disease Acquired, in particular its incidence reaches a Approximately 9 to 19 cases per 100,000 children under the age of 5 (Centers for Disease Control and Prevention, 2016).
Specifically, in 2000, some 4248 hospitalizations associated with Kawasaki disease occurred in medical centers (Centers for Disease Control and Prevention, 2016).
Characteristic signs and symptoms
Kawasaki disease is considered a multisystemic pathology, due mainly to the variability of areas and bodily organs that can Be affected by the inflammation of the circulatory system and consequently, by the reduction of efficient blood flow. However, some of the Most commonly include (Bou, 2014, Delgado Rubio, 2016, Prego Petit, 2003):
to) Cardiac Manifestations
As we have indicated previously, Kawasaki disease is a type of vasculitis, so the cardinal medical complication of this disorder is Inflammation of the walls of blood vessels.
Specifically, this pathology affects the median arteries as a priority, although it is also possible to observe a significant alteration of the Blood vessels of small and medium caliber, including arteries, capillaries, and veins. In addition, one of the most affected arteries Is the Coronary , So the probability of developing a myocardial infarction is high.
In addition, within the cardiac manifestations some of the most frequent are:
- Myocarditis : In this case, the term myocarditis is used to refer to the presence of significant inflammation of the Myocardium or cardiac muscle area, as a consequence, can appear diverse manifestations as the arrhythmia or the heart failure.
- Pericarditis : In this case, the term Pericarditis Is used to refer to the presence of significant inflammation of the Pericardium or of the tissue that covers the cardiac structures, consequently, different secondary manifestations can appear like the infarct of Myocardium or myocarditis.
- Valvulitis : In this case, the term valvulitis is used to refer to the presence of a significant inflammation of the cardiac valves (mitral, aortic, pulmonary and tricuspid), responsible for controlling the direction of blood flow. As a consequence, several pathologies can appear such as valvulopathy, valvular stenosis, valvular insufficiency or endocarditis.
- Aneurysms : In this case, the term aneurysm Is used to refer to the development of a weak or thinner area on the walls Of the blood vessels. With blood flow, the weakened area may acquire a sac shape, protruding and thus with a high probability Rupture and bleeding in any area of the body. Specifically, one of the most serious types are those that appear in the cerebral circulatory system, By the possibility of hemorrhage and mechanical pressure in nerve areas.
B) Ocular impairment
Ophthalmologic manifestations are one of the most common medical findings in Kawasaki disease, some of which are characterized For the development of:
- Conjunctival injection and hyperthermia: The presence of a reddening of the ocular areas, simulating the presence of lines or points of blood material is a common finding in Kawasaki disease. In addition, this type of alterations can produce episodes of ocular pain or loss of the visual capacity.
- Conjunctivitis: In this case the term conjunctivitis is used to refer to the presence of significant inflammation of the transparent tissue covering both the eyeball and the inside of the eyelid. As a consequence, various pathologies related to eye redness, inflammation or episodes of pain may appear.
C) Fever
The pathological increase in body temperature is one of the earliest medical symptoms of Kawasaki disease.
At a clinical level, body temperature is extremely high, in some cases it can reach 40 ° C or even higher temperatures.
Although it occurs in recurrent episodes and responds to pharmacological treatment, body temperature tends to remain around 38 degrees.
Specifically, the duration of fever episodes if no type of therapeutic approach is used is usually about two weeks, however, Can reach a longer duration, around 4 weeks.
(D) Skin manifestations
An abnormality in blood circulation can also affect skin and musculoskeletal integrity. Some of the most common pathological findings in Kawasaki disease include:
- Erythema cutaneous : In this case, the term erythema is used to refer to the presence of inflammation and focal skin redness of some cutaneous areas. It usually affects the limbs, especially the hands and feet.
- Edema in the extremities: This pathology is a type of cutaneous disorder that manifests as an inflammation and redness that is usually accompanied by the development of protuberances, pustules and fever.
- Skin peeling: In this case, this cutaneous pathology is characterized by the loss or detachment of the outermost skin layers. It is usually superficial and spontaneous and mainly affects the fingers.
E) Oral manifestations
One of the most characteristic findings of Kawasaki disease is oral involvement, some pathologies include:
- Cleft lip: A significant dryness and bleeding of the buccal structures is observed, especially accentuated before The physical contact.
- Aframbuesada language: The tongue usually presents an inflamed appearance, taking a darker color than usual, with a size Increased taste buds.
F) Lymphatic manifestations
The lymphatic system is essentially responsible for transporting the lymph from the circulatory system to the different caporal organs.
The lymph Is an aqueous substance that comes from the cardiac material and whose main function is to act as an intermediary in the Nutrient exchanges.
Thus, in Kawasaki disease, the presence of anomalies in this system, has the consequence of developing some pathologies, such as the adenoma cervical.
Specifically, in Kawasaki disease, it is common to detect the development of cervical lymphadenopathy, that is, an abnormal swelling of the lymph nodes Lymphatics Which are found at the cervical level.
G) Neurological manifestations
Although the affectation of the nervous system is less common than the previous ones, in many cases can be detected diverse alterations and pathologies Neurological disorders, including meningitis or cerebral aneurysms.
Clinical course
In the typical clinical evolution of Kawasaki disease three fundamental phases can be distinguished (Delgado Rubio, 2016):
- Acute or febrile phase: The first phase of the disease usually lasts about one to two weeks. It is usually characterized by the presence of febrile episodes, adenopathy, conjunctive injection, erythema in the hands and feet, oral lesions, meningitis, and the development of other disorders related to irritability and anorexia.
- Subacute phase : After the first two weeks have passed, some of the above symptoms can be maintained, especially Ocular involvement or adenopathy and, in addition, new ones such as desquamation are developed. On the other hand, some complications Secondary medical conditions including myocardial infarctions or aneurysms.
- Recovery phase or convalescence : In the final phase of Kawasaki disease the clinical manifestations tend to remit. However, some complications may appear recurrent or require a longer time to resolve, such as aneurysms.
Causes
At present, the specific etiologic cause of Kawasaki disease has not yet been identified (American Heart Association, 2015).
Genetic and hereditary patterns have not been observed; however, their clinical course seems to suggest the presence of infectious etiological factors or (American Heart Association, 2015).
Some of the current hypotheses suggest that the presence of an infectious episode, probably respiratory by a viral agent, has as Consequently the development of a vascular inflammatory process in genetically predisposed people (Boraveli and Chiaverini, 2014).
Diagnosis
The diagnosis of Kawasaki disease is primarily clinical, and is based on the following criteria (Delgado Rubio, 2016):
1. Presence of fever for 5 days or more.
2. Presence of conjunctival injection
3. Presence of cervical lymphadenopathy.
4. Presence of skin rash.
5. Presence of labial and buccal anomalies (dry, red, fissured lips, pharyngeal erythema, aframatose tongue)
In addition, laboratory tests are usually used to confirm the diagnosis and rule out other medical conditions: echocardiogram, Blood, magnetic resonance , Urinalysis, cerebrospinal fluid extraction, etc. (Bou, 2014).
Treatment
Kawasaki disease usually responds favorably to treatments prescribed by health professionals (Boraveli and Chiaverini, 2014).
Usually the initial treatment usually lasts about 5 days, however it can be prolonged if the medical complication persists. It usually uses the intravenous administration of immunoglobulin and acetylsalicylic acid (aspirin) (Boraveli and Chiaverini, 2014).
If symptoms continue, parallel administration of corticosteroids, calcineurin inhibitors or plasma exchanges (Boraveli And Chiaverini, 2014).
References
- AHA. (2016). Kawasaki Disease . Retrieved from the American Heart Association.
- Arias Cabello, B., Ferández Álvarez, H., & Ordaz Favila, J. (2016). Ocular manifestations in Kawasaki disease. Experience in the Institute National Pediatrics. Rev. Mex. Oftalmol , 3-8.
- Boralevi, F., & Chiaverini, C. (2014). Kawasaki disease. EMC .
- Bou, R. (2014). Kawasaki disease. AEP , 117-129.
- CDC. (2013). About Kawasaki Disease . Retrieved from"Center for Disease Control and Prevention."
- Delgado Rubio, A. (2016). Kawasaki disease. Diagnostic and therapeutic protocols in pediatrics , 21-26.
- García Rodríguez, F., Flores Pineda, A., Villareal Treviño, A., Salinas Encinas, D., Lara Heredia, P., Maldonado Vázquez, M.,. . . Faugier Fuentes, E. (2016). Kawasaki disease in a pediatric hospital in Mexico. Bol Med Hosp Infant Mex .
- KDF. (2016). What is Kawasaki Disease? Retrieved from the Kawasaki Disease Foundation.
- MayoClinic. (2014). Kawasaki disease . Obtained from MayoClinic.
- Melbourne, T.R. (2016). Kawasaki Disease . Retrieved from The Royal Children's Hospital Melbourne.
- NIH. (2016). Kawasaki disease . Obtained from MedlinePlus.
- Prego Petit, J. (2003). Kawasaki disease. Arch Pediatr Urug .
- Sotelo-Cruz, N. (2016). Atypical or incomplete course of Kawasaki disease. Bol Med Hosp Infant Mex .
- Image source .