Kawasaki disease , also known as mucocutaneous lymph node syndrome , is a disease in which blood vessels throughout the body become inflamed. The most common symptoms include fever that lasts for more than five days unaffected by regular drugs, large lymph nodes in the neck, rashes in the genital area, and red eyes, lips, palms or soles. Other symptoms include sore throat and diarrhea. Within three weeks of the onset of symptoms, the skin from the hands and feet can be peeled off. Recovery usually occurs. In some children, coronary artery aneurysms can form in the heart after 1-2 years.
The cause is unknown. This may be caused by an infection that triggers an autoimmune response in those who are genetically susceptible. It does not spread among people. Diagnosis is usually based on signs and symptoms of a person. Other tests such as cardiac ultrasound and blood tests may support the diagnosis. Other conditions that may present the same include dengue fever and juvenile rheumatoid arthritis.
Initially treatment is usually with high doses of aspirin and immunoglobulin. Usually with treatment, the fever heals within 24 hours and there is full recovery. If the coronary artery is involved, ongoing care or surgery is sometimes necessary. Without treatment, coronary artery problems occur up to 25% and about 1% die. With treatment, the risk of death was 0.17%.
Kawasaki's disease is rare. It affects between 8 and 67 per 100,000 people under the age of five except in Japan where it affects 124 per 100,000. This is much less common after the age of five. Boys are more affected than girls. This disorder was first described in 1967 by Tomisaku Kawasaki in Japan.
Video Kawasaki disease
Signs and symptoms
Kawasaki's disease often begins with a high and persistent fever that is not very responsive to normal treatment with paracetamol (acetaminophen) or ibuprofen. This is the most prominent symptom in Kawasaki disease, is a characteristic sign of the acute phase of the disease, usually high (above 39-40Ã, Â ° C), remittent, and followed by extreme irritability. More recently, reportedly present in patients with atypical or incomplete Kawasaki disease; However, it does not exist in 100% of cases. The first day of fever is considered to be the first day of the disease, and the duration of fever is an average of one to two weeks; in the absence of treatment, this can extend for three to four weeks. Prolonged fever is associated with higher incidence of cardiac involvement. It responds to some antipyretic drugs and does not stop with the introduction of antibiotics. However, when proper therapy is started - intravenous immunoglobulin and aspirin - fever disappears after two days.
Bilateral conjunctival inflammation is reported to be the most common symptom after a fever. This usually involves bulbar conjunctiva, not accompanied by pus, and is not painful. It usually begins shortly after the onset of fever during the acute stage of the disease. Anterior uveitis can be found on slit-lamp examination. Iritis can happen as well. Keratic precipitate is another eye manifestation (can be detected by slit lamp but usually too small to be seen by the naked eye).
Kawasaki disease presents with mouth symptoms, the most distinctive changes are red tongue, swollen lips with vertical fractures and bleeding. The mouth and throat mucosa may be bright red, and the tongue may have a distinctive appearance of "strawberry tongue" (marked reddish with prominent guilla papillae). These oral symptoms are caused by a typical necrotizing microvasculitis with fibrinoid necrosis.
Cervical lymphadenopathy is seen in 50% to 75% of people, while other features are estimated to occur in 90% of patients, but can sometimes be the dominant symptom. According to the definition of diagnostic criteria, at least one lymph node is disturbed> = 15 mm diameter should be involved. The affected lymph nodes do not cause pain or minimal pain, are non-fluctuating, and nonsupurative; erythema of neighboring skin may occur. Children with fever and neck adenitis who do not respond to antibiotics should have Kawasaki disease considered part of the differential diagnosis.
In the acute phase of the disease, peripheral limb changes may include erythema of the palms and soles of the feet, often prominent with sharp demarcation and often accompanied by strong muscular edema of the dorsa of the hands or feet. This is why affected children often refuse to hold objects in their hands or to hold a load on their legs. Then, during the healing phase or subacute phase, desquamation of the fingers and toes usually begins in periungual areas within two to three weeks after the onset of fever and may extend to include the palms of the hands and soles of the feet. Approximately 11% of children affected by this disease can continue to peel the skin for years. One to two months after the onset of a fever, transverse grooves across the nail may develop (Beau lines), and sometimes nails are shed.
The most common skin manifestation is a diffuse macular erythematous-papular rash, which is quite non-specific. The rash varies over time and is characteristically located in the trunk; it can be more diffuse to involve the face, extremities, and perineum. Many other forms of skin lesions have been reported; they may include scarlatiniform, papular, urticariform, erythema multiform-like, and purpura lesions; even micropustules are reported. It can be polymorphic, not itchy, and usually observed until the fifth day of fever. However, it is never bullous or vesicular.
In the acute stage of Kawasaki's disease, systemic inflammatory changes are evident in many organs. Joint pain (arthralgia) and swelling, often symmetrical, and arthritis may also occur. Myocarditis, diarrhea, pericarditis, valvulitis, aseptic meningitis, pneumonitis, lymphadenitis, and hepatitis may be present and manifested by the presence of inflammatory cells in the affected tissue. If left untreated, some symptoms will eventually succumb, but coronary artery aneurysms will not improve, resulting in significant death or disability risk due to myocardial infarction. If treated quickly, these risks can be avoided and the course of the disease is reduced.
Other reported nonspecific symptoms include cough, rhinorrhea, sputum, vomiting, headache, and seizures.
The course of the disease can be divided into three clinical phases. Acute phase phase, usually lasting for one to two weeks, is characterized by fever, conjunctival injection, oral mucosal erythema, erythema and swelling of the hands and feet, rash, cervical adenopathy, aseptic meningitis, diarrhea, and dysfunction heart. Myocarditis often occurs during this time, and pericardial effusion can be found. Coronary arteritis may be present, but aneurysms are not generally seen by echocardiography.
Presentation between adults and children is different, since adult neck lymph nodes are more affected (93% of adults versus 15% of children), hepatitis (65% versus 10%), and arthralgia (61% versus 24-38 %). Some people have atypical unusual and may not have classical symptoms. This occurs especially in young infants; those people are especially at increased risk of cardiac artery aneurysm.
Cardiac
Cardiac complications are the most important aspect of Kawasaki's disease. This is the leading cause of heart disease acquired in childhood in the United States and Japan. In developed countries, it appears to have replaced acute rheumatic fever as the most common cause of heart disease acquired in children. Coronary artery aneurysm occurs as a sequel to vasculitis in 20-25% of untreated children. It was first detected in an average of 10 days of disease and the peak frequency of dilation of the coronary artery or aneurysm occurred within four weeks of onset. Aneurysms are classified into small (internal diameter of the vessel wall & lt; 5 mm), medium (5 to 8 mm in diameter), and giant (diameter & gt; 8 mm). Sausal and fusiform aneurysms usually develop between 18 and 25 days after the onset of the disease.
Even when treated with high-dose IVIG regimens within the first 10 days of the disease, 5% of children with Kawasaki disease develop in minimal coronary artery dilation and 1% develop giant aneurysms. Death may occur due to myocardial infarction secondary to the formation of blood clots on coronary artery aneurysm or rupture of large coronary artery aneurysm. The most common death occurs two to 12 weeks after the onset of the disease.
Many risk factors that predict coronary artery aneurysm have been identified, including persistent fever after IVIG therapy, low hemoglobin concentration, low albumin concentration, high white blood cell count, high band count, high CRP concentration, male gender, and less age one. year. Coronary artery lesions due to Kawasaki disease change dynamically over time. A resolution of one to two years after the onset of the disease has been observed in half the blood vessels with coronary aneurysms. Narrowing of the coronary arteries, which occurs as a result of the healing process of blood vessel walls, often causes significant obstruction of the blood vessels and causes the heart to not receive enough blood and oxygen. This can eventually lead to death of the heart muscle tissue (myocardial infarction).
MI is caused by thrombotic occlusion in the coronary artery aneurysms, stenotik, or coronary artery aneurysms and stenosis is the leading cause of death due to Kawasaki disease. The highest risk of MI occurs in the first year after the onset of the disease. MI in children has different symptoms than adults. The main symptoms are shock, anxiety, vomiting, and abdominal pain; Chest pain is most common in older children. Most of these children experience attacks that occur during sleep or at rest, and about one-third of attacks show no symptoms.
Valve insufficiency, especially mitral or tricuspid valves, is often observed in the acute phase of Kawasaki's disease due to inflammation of the heart valve or inflammation in myocardial dysfunction caused by the heart muscle, regardless of coronary involvement. These lesions largely disappear with the resolution of acute illness, but a group of very small lesions survive and develop. There is also a slow-onset aortic or mitral insufficiency caused by thickening or deformation of the fibrosed valve, with time from several months to years after the onset of Kawasaki's disease. Some of these lesions require valve replacement.
More
Other complications of Kawasaki disease have been described, such as other arterial aneurysms: aortic aneurysms, with a higher reported number of cases involving abdominal aorta, axillary artery aneurysm, brachiocephalic artery aneurysm, iliac artery aneurysm and femoral artery, and renal artery aneurysm. Other vascular complications may occur such as increased wall thickness and decreased distensibility of the carotid artery, aorta, and brachioradial artery. This vascular tone changes secondary to endothelial dysfunction. In addition, children with Kawasaki disease, with or without coronary artery complications, may have a worse cardiovascular risk profile, such as high blood pressure, obesity, and abnormal serum lipid profiles.
Gastrointestinal complications in Kawasaki disease are similar to those observed in Henoch-SchÃÆ'¶nlein purpura, such as: intestinal obstruction, intestinal swelling, intestinal ischemia, pseudo bowel obstruction, and acute abdomen.
Eye-related changes have been described since the 1980s, found as uveitis, iridocyclitis, conjunctival bleeding, optic neuritis, amaurosis, and ocular artery obstruction. It can also be found as necrotizing vasculitis, developing into peripheral gangrene.
Neurologic complications per central nervous system lesion are increasingly reported. The neurological complications found are meningoencephalitis, subdural effusion, cerebral hypoperfusion, cerebral ischemia and infarction, cerebral infarction, manifestation with seizures, chorea, hemiplegia, mental confusion, lethargy and coma, or even cerebral infarction without neurologic manifestations. Other neurologic complications of cranial nerve involvement are reported as ataxia, facial palsy, and sensorineural hearing loss. Behavioral changes are thought to be caused by local cerebral hypoperfusion, may include attention deficit, learning deficit, emotional disturbance (emotional lability, fear of night, and night terrors), and internalization problems (anxiety, depression or aggressive behavior).
Maps Kawasaki disease
Cause
Because the cause of Kawasaki disease is still unknown, the disease is more accurately referred to as Kawasaki syndrome. The causes are widely hypothesized to involve the interaction of genetic and environmental factors, possibly including infection in combination with a genetic predisposition to the autoimmune mechanism. The specific cause is unknown, but current theories are mainly centered on immunological causes. The evidence is increasingly pointing to the cause of the infection, but the debate continues whether the cause is a conventional antigenic substance or superantigen. Researchers at Boston Children's Hospital report, "several studies have found a link between the onset of Kawasaki disease and recent exposure to carpet or residential cleansing near stagnant water bodies, but the cause and effect has not been established."
Other data show a clear correlation between Kawasaki's disease and troposphere wind patterns; the wind blowing from Central Asia correlates with cases of Kawasaki disease in Japan, Hawaii, and San Diego. This association with tropospheric wind has been shown to be modulated on a seasonal and interannual time scale by the phenomenon of Southern El Nià ± ous Oscillation, which further indicates that the agent responsible for the disease is a pathogen carried by the wind. Efforts are being made to identify suspected pathogens in air filters that are flown at altitudes above Japan.
An association has been identified with the SNP in the ITPKC gene , which encodes an enzyme that negatively regulates T-cell activation. Regardless of where they live, Japanese children are more likely than other children to manifest the disease, which shows genetic susceptibility. Serotypes of HLA-B51 have been found to be associated with disease endemic events.
Diagnosis
Kawasaki's disease can only be diagnosed clinically (ie, with medical signs and symptoms). There are no special laboratory tests for this condition. It is difficult to make a diagnosis, especially early in the course of the disease, and often children are not diagnosed until they have seen some health care providers. Many other serious diseases can cause similar symptoms, and should be considered in the differential diagnosis, including dengue fever, toxic shock syndrome, juvenile idiopathic arthritis, and childhood mercury poisoning (acrodynia infantil).
Classically, five days of fever plus four of the five diagnostic criteria must be met for diagnosis. These criteria are:
- erythema of the lips or the oral cavity or the rupture of the lips
- rash in the trunk
- swelling or erythema in the hands or feet
- red eye (conjunctival injection)
- a swollen lymph node at the neck of at least 15 mm
Many children, especially infants, who are finally diagnosed with Kawasaki disease, do not show all of the above criteria. In fact, many experts now recommend treatment for Kawasaki's disease even if only three days have passed and there are at least three diagnostic criteria, especially if other tests reveal abnormalities consistent with Kawasaki's disease. In addition, the diagnosis can be done purely by detecting coronary artery aneurysm in the proper clinical setting.
Investigation
A physical check will show many of the features listed above.
Blood test
- A complete blood count may reveal normocytic anemia and ultimately thrombocytosis.
- The erythrocyte sedimentation rate will increase.
- C-reactive protein will increase.
- Liver function tests may show evidence of liver inflammation and low serum albumin levels.
Other optional tests include:
- Electrocardiogram may show evidence of ventricular dysfunction or, occasionally, arrhythmias due to myocarditis.
- The echocardiogram may show subtle coronary artery changes or, later, the actual aneurysm.
- Ultrasound or computerized tomography can show the hydrops (enlargement) of the gallbladder.
- Urinalysis may show white blood cells and proteins in the urine (pyuria and proteinuria) without evidence of bacterial growth.
- The lumbar puncture may show evidence of aseptic meningitis.
- Angiography has historically been used to detect coronary artery aneurysms, and remains a gold standard for their detection, but is rarely used today unless coronary artery aneurysms have been detected by echocardiography.
- Temporal artery biopsy
Classification
Inflammation, or vasculitis of the arteries and veins occurs throughout the body. This is usually caused by increased production of immune system cells against pathogens, or autoimmunity. Systemic vasculitides can be classified according to the type of cells involved in proliferation, as well as the type of specific tissue damage that occurs inside the blood vessels or artery walls. Under this classification scheme for systemic vasculitis, Kawasaki's disease is considered a necrotizing vasculitis (also called necrotizing angiitis), which can be identified histologically by the occurrence of necrosis (tissue death), fibrosis, and cell proliferation associated with inflammation in the inner lining of the wall blood vessel.
Other diseases that show necrotizing vasculitis include polyarteritis nodosa, granulomatosis with polyangiitis (GPA), Henoch-SchÃÆ'¶nlein purpura and eosinophilic granulomatosis with polyangiitis (EGPA).
Kawasaki's disease can be further classified as a medium-sized vasculitis, affecting small and medium-sized blood vessels, such as smaller blood vessels (veins and arteries in the skin) ranging from 50 to 100 Ã, μm in diameter. Kawasaki's disease is also considered a primary childhood vasculitis, a disorder associated with vasculitis that primarily affects children under the age of 18. A recent vasculitide-based evaluation based on consensus on children resulted in a classification scheme for this disorder, differentiating them and suggesting a more concrete set of diagnostic criteria for each. In the classification of this childhood vasculitid, Kawasaki's disease is, once again, a medium-sized vasculitic vessel.
It is also an autoimmune vasculitis form, and is not associated with ANCA antibodies, unlike other vasculitic disorders associated with it (such as granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis). This categorization is considered essential for proper care.
Treatment
Children with Kawasaki disease should be admitted to the hospital and treated by a doctor who has experience with the disease. While in the academic medical center, care is often shared between pediatric cardiology, pediatric rheumatology, and pediatric infectious disease specialists (although no specific infectious agent has been identified). To prevent damage to the coronary arteries, treatment should begin as soon as the diagnosis is made.
Intravenous immunoglobulin (IVIG) is a standard treatment for Kawasaki disease and is given in high doses with marked improvement typically recorded within 24 hours. If the fever does not respond, additional doses may have to be considered. In rare cases, a third dose may be given to the child. IVIG itself is most useful in the first seven days of fever onset, in terms of preventing coronary artery aneurysm.
Salicylic therapy, especially aspirin, remains an important part of treatment (although questioned by some) but salicylate alone is not as effective as IVIG. Aspirin therapy starts at high doses until the fever subsides, and then continues with a low dose when the patient returns home, usually for two months to prevent blood clots. Except for Kawasaki's disease and some other indications, aspirin is not usually recommended for children because of its association with Reye's syndrome. Since children with Kawasaki's disease will take aspirin for several months, vaccination against varicella and influenza is needed, as this infection is most likely to cause Reye's syndrome. High doses of aspirin are associated with anemia and do not benefit disease outcomes.
Corticosteroids have also been used, especially when other treatments fail or symptoms recur, but in randomized controlled trials, the addition of corticosteroids to the immune globulin and aspirin does not improve yield. In addition, the use of corticosteroids in Kawasaki disease settings is associated with an increased risk of coronary artery aneurysm, so its use is generally contraindicated in this setting. In the case of refractory Kawasaki disease against IVIG, cyclophosphamide and plasma exchange have been investigated as possible treatment, with varying outcomes.
Prognosis
With early treatment, rapid recovery of acute symptoms can be expected, and the risk of coronary artery aneurysms is greatly reduced. Untreated, the acute symptoms of Kawasaki's disease are limited to the patient's (i.e.) endurance), but the risk of involvement of the coronary artery is much greater. Overall, about 2% of patients die from complications of coronary vasculitis.
Laboratory evidence of increased inflammation combined with demographic features (male sex, age less than six months or more than eight years) and an incomplete response to IVIG therapy make the profile of high-risk patients with Kawasaki disease. The likelihood that an aneurysm will resolve appears to be determined in large size by its initial size, where smaller aneurysms have a greater likelihood of regression. Other factors are positively associated with aneurysm regression, including those younger than one year at the onset of Kawasaki's disease, fusiform rather than the morphology of the sacral aneurysm, and the location of aneurysms in the distal coronary segment. The highest rate of development to stenosis occurs among those who develop large aneurysms. The worst prognosis occurs in children with giant aneurysms. These severe results may require further treatment such as percutaneous transluminal angioplasty, coronary artery stenting, bypass grafting, and even heart transplantation.
A recurrence of symptoms may occur immediately after initial treatment with IVIG. This usually requires rehospitalization and retreatation. Treatment with IVIG may cause acute allergic and nonallergic reactions, aseptic meningitis, excess fluids and, rarely, other serious reactions. Overall, life-threatening complications resulting from therapy for Kawasaki's disease are very rare, especially compared to the risk of nontreatment. Also, evidence suggests Kawasaki's disease results in changes in lipid metabolism that persist beyond the clinical resolution of the disease.
Rarely, recurrence can occur in Kawasaki disease with or without treatment.
Epidemiology
Kawasaki's disease affects boys more than women, with people from Asian ethnicity, particularly Japanese and Korean, most vulnerable, as well as people of Afro-Caribbean ethnicity. This disease has rarely occurred in the Caucasus until the last few decades, and the incidence rate fluctuates from country to country.
Currently, Kawasaki disease is the most commonly diagnosed pediatric vasculitis in the world. So far, the highest incidence of Kawasaki disease occurred in Japan, with recent research putting the attack rate at 218.6 per 100,000 children & lt; 5 years (about one in 450 children). At the current attack rate, more than one in 150 children in Japan will develop Kawasaki disease during their lifetime.
However, his incident in the United States increased. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than five years. About 2,000-4,000 cases are identified in the US each year (9 to 19 per 100,000 children younger than 5 years).
In the UK, estimates of incidence rates vary due to the scarcity of Kawasaki disease. However, it is believed to affect less than one in every 25,000 people. The incidence of disease doubled from 1991 to 2000, however, with four cases per 100,000 children in 1991 compared with an increase of eight cases per 100,000 in 2000.
In the continent of the United States, Kawasaki's disease occurs more frequently during winter and early spring, boys with diseases than girls with 1.5-1.7: 1, and 76% of affected children are & lt; 5 years.
History
The disease was first reported by Tomisaku Kawasaki to a four-year-old child with a rash and fever at the Red Cross Hospital in Tokyo in January 1961, and he then published a report on 50 similar cases. Later, Kawasaki and colleagues were convinced of a definite cardiac involvement when they studied and reported 23 cases, of which 11 (48%) patients had abnormalities detected by the electrocardiogram. In 1974, the first description of the disorder was published in English literature. In 1976, Melish et al. described the same disease in 16 children in Hawaii. Melish and Kawasaki independently developed the same diagnostic criteria for disorders, which are still in use today to make the diagnosis of classic Kawasaki disease.
A question arises whether the disease started only during the period between 1960 and 1970, but then the preserved heart of a seven-year-old boy who died in 1870 was examined and showed three aneurysms of coronary arteries with freezing, as well as consistent pathological changes with Kawasaki's disease. Kawasaki's disease is now recognized worldwide. In the United States and other developed countries, it appears to have replaced acute rheumatic fever as the most common cause of heart disease acquired in children.
References
External links
- Kawasaki Disease - Stanford Children's Health
- Kawasaki disease research program
- the Kawasaki disease foundation
- Kawasaki disease information from the Heart Center of Seattle Children's Hospital
- http://www.kawasaki-disease-taiwan.com/- Kawasaki Disease Taiwan.
Source of the article : Wikipedia
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