Kawasaki disease in a child. Signs of Kawasaki syndrome in children and methods of treating pathology. Complete Kawasaki syndrome is characterized by the following symptoms:

Kawasaki disease is a rare but rather dangerous vascular pathology that belongs to the category of systemic vasculitis. During the course of such a disease, an attack on the walls of blood vessels by cells of one's own immune system is noted. At present, the causes of the formation of such an ailment remain a blank spot for clinicians, but it is generally accepted that pathogenic bacteria and viruses have pathological effects.

This syndrome has specific clinical signs, including prolonged fever, enlarged lymph nodes, changes in the organs of vision and tongue, as well as swelling of the palms and feet.

The diagnosis is established by examining the symptomatic picture, as well as through the implementation of a wide range of laboratory and instrumental examinations. Such manipulations are necessarily supplemented by a thorough examination of the patient.

Treatment of the disease is carried out only by conservative methods, the essence of which is the reception, intravenous administration and local use of medicines.

The international classification of diseases of the tenth revision allocates a separate code for such a syndrome. ICD-10 code - M30.03.

Etiology

To date, it is not possible to find out why Kawasaki syndrome develops, however, clinicians from the field of pediatrics and pediatric rheumatology distinguish several theories, according to which the following can serve as a predisposing factor:

• various autoimmune processes, during which the immune system begins to produce antibodies against its own cells, tissues, blood vessels and internal organs;
• penetration into the human body of pathogenic agents;
• hereditary predisposition - the probability that the offspring of patients with a similar diagnosis will suffer from such an ailment is approximately 9%.

In the vast majority of situations, pathological microorganisms are:

• retroviruses;
• parvoviruses.

It is also worth noting about the main risk group, which consists of:

• male representatives;
• children suffering from immunodeficiency conditions;
• babies in whose families cases of diagnosing Kawasaki disease have already been recorded;
• persons belonging to the yellow race - most often outbreaks of such an ailment are recorded among the Japanese;
• children from 1 year to 5 years.

Clinicians distinguish two main peaks of incidence:

• the first - in the age category from 9 to 11 months;
• the second - from 1.5 to 2 years.

It is noteworthy that Kawasaki disease in adults and children older than 5 years is almost never diagnosed.

Classification

During its progression, Kawasaki syndrome goes through several stages of development:

• acute - lasts from one week to 10 days;
• subacute - can take from 14 to 21 days;
• recovery phase - the duration is from 2 months to 2 years.

In addition, it is customary to distinguish a complete and incomplete version of the course of such a pathology.

Symptoms

A distinctive feature of such an ailment is that the symptoms appear suddenly and abruptly, and the first clinical sign is an increase in body temperature to 38-40 degrees. Often the febrile period is 2 weeks. Nevertheless, an increase in the duration of the course of such a state is considered a sign leading to an unfavorable prognosis.

In a typical course of Kawasaki disease, the symptoms will be as follows:

• persistent swelling of the face and limbs;
• reddening of the skin of the palms and feet, several times less often it takes on a purple color;
• the formation of small flat spots with a red tint. Often, such rashes are localized in the groin area, as well as on the upper and lower extremities;
• the occurrence of blisters on the skin by type;
• peeling of the skin;
• inflammation of the choroid of the organs of vision;
• bilateral ;
• dry lips, leading to cracking and bleeding;
• enlargement of the tonsils;
• change in the size of the lymph nodes in a big direction - most often the cervical lymph nodes are involved in the pathology;
• swelling of the tongue, while it acquires a bright red hue;
• pain in the heart and abdomen;
• joint thickening;
• bouts of nausea accompanied by vomiting;
• stool disorder, expressed in the alternation of constipation and diarrhea;
• sleep disturbance, up to insomnia;
• fatigue and loss of strength;
• and shortness of breath;
• joint pain.

Kawasaki syndrome in infants is expressed in:

• increased excitability;
• sleep disorders;
• severe skin itching;
• refusal to eat;
• profuse diarrhea;
• frequent vomiting.

Symptoms of an incomplete syndrome are presented:

• fever lasting less than a week;
• feet and palms;
• skin rashes;
• cracked lips, but without bleeding;
• swelling of the tongue;
• conjunctivitis.

Diagnostics

Due to the fact that such a pathology has pronounced specific signs, there are no problems with establishing the correct diagnosis. Nevertheless, diagnostics must necessarily be a comprehensive approach.

From this it follows that, first of all, the clinician needs:

• get acquainted with the medical history - to identify immunodeficiency or autoimmune conditions that can provoke the manifestation of this syndrome;
• collect and analyze the history of life - this should include information regarding the diagnosis of such an ailment among close relatives of a small patient;
• conduct a thorough examination of the patient, with a mandatory study of the condition of the skin, measurement of heart rate and temperature;
• to interrogate the patient's parents in detail about the first time of occurrence and the intensity of the severity of clinical manifestations. This will enable the doctor to compile a complete symptomatic picture and understand the nature of the course of Kawasaki syndrome.

To clarify the diagnosis, the following laboratory tests are necessary:

• general clinical blood test;
• immunological tests;
• serological tests;
• blood biochemistry;
• general analysis of urine.

As for instrumental examinations, the following have the greatest diagnostic value:

• radiography of the sternum;
• ECG and echocardiography;
• daily ECG monitoring;
• ultrasonography of internal organs;
• CT and MRI;
• coronary angiography;
• lumbar puncture.

During differential diagnosis, Kawasaki disease in children is differentiated from:

• scarlet fever and;
• Steven-Johnson syndrome.

Treatment

After confirming the diagnosis, the patient should be hospitalized. In addition to the therapist and rheumatologist, a cardiologist and a cardiac surgeon take part in the treatment. Therapy is carried out only by conservative methods and is aimed at:

• relief of a feverish state;
• stabilization of immune responses;
• prevention of complications.

To achieve these results, use:

• immunoglobulins that are administered intravenously - such substances have the greatest effect if they are used in the first 10 days from the onset of the disease;
• anti-inflammatory drugs - take a course of 5-10 days;
• medicines aimed at thinning the blood;
• anticoagulants - their injection is shown;
• preparations of acetylsalicylic acid;
• corticosteroid and hormonal ointments - to eliminate skin manifestations.

Surgical intervention may be required only in cases of formation of consequences. The issue of surgery is decided for each patient individually.

Possible Complications

Despite the fact that the outcome of such an ailment is often favorable, Kawasaki syndrome can lead to the development of such complications:

• inflammation of the joints;
• and meningitis;
• and urethritis;
• and valvulitis;
• upper or .

Prevention and prognosis

Against the background of the fact that the causes of the development of Kawasaki disease in a child remain unknown, it is quite natural that there are no specific preventive measures. Nevertheless, to prevent the occurrence of this disease, the following clinical recommendations are used:

• permanent strengthening of the immune system;
• vitamin therapy and rational nutrition;
• control over the adequate course of pregnancy;
• counseling with a geneticist - shown to couples in whose families such a syndrome was previously diagnosed;
• treatment in the early stages of the progression of diseases of a viral and bacterial nature;
• regular examination of the baby by a pediatrician and other children's specialists.

Timely provision of qualified and adequate assistance is the key to a favorable prognosis. However, it should be noted that complications occur in every 5 patients with a similar diagnosis. The risk of lethality varies from 0.8 to 3%.

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Kawasaki disease, also known as Kawasaki syndrome, mucocutaneous lymph node syndrome, and mucocutaneous lymph node syndrome, is an autoimmune disease in which medium-sized blood vessels throughout the body become inflamed. It is often seen in children under the age of five. It affects many organ systems, mainly which include the blood vessels, skin, mucous membranes, and lymph nodes. It affects the heart less frequently, but more severely, where it can cause fatal coronary artery aneurysms in untreated children. Without treatment, mortality can be as high as 1%, usually within six weeks of onset. With treatment, the mortality rate is 0.17% in the US. Often a prior viral infection may play a role in the pathogenesis of the disease. The skin, conjunctiva of the eyes, and mucous membranes of the mouth become red and inflamed. Swelling of the hands and feet is common, and the lymph nodes in the neck are often enlarged. Intermittent fever, often 37.8 °C (100.0 °F) or higher, is a feature of the acute phase of the disease. In untreated children, fever lasts about 10 days, but can range from five to 25 days. The disease was first described in 1967 by Tomisaku Kawasaki in Japan.

Classification

Systemic vasculitis is an inflammatory disease affecting arteries and veins throughout the body, usually caused by cell proliferation associated with an immune response to a pathogen or an autoimmune reaction. Systemic vasculitis can be classified according to the type of cells involved in the proliferation as well as the specific type of tissue damage seen in the walls of arteries and veins. According to this classification scheme for systemic vasculitis, Kawasaki disease is considered necrotizing vasculitis (also called necrotizing angiitis), which can be defined histologically by the appearance of necrosis (tissue death), fibrosis, and cell proliferation associated with inflammation of the inner layer of the vascular wall. (Other conditions suggestive of necrotizing vasculitis include polyarteritis nodosa, granulomatosis with polyangiitis, Henoch-Schonlein purpura, and Churg-Strauss syndrome.) Kawasaki disease can be further classified as a medium-sized vessel vasculitis affecting medium to small blood vessels such as as a small cutaneous vasculature (veins and arteries in the skin), which are 50 to 100 microns in diameter. Kawasaki disease is also considered primary childhood vasculitis, a vasculitis-related disease that primarily affects children under 18 years of age. Recently, the opinion-based assessment of vasculitis, which occurs predominantly in children, has led to a classification scheme for these diseases to distinguish between them, which provides a more accurate set of diagnostic criteria for each. Under this classification of childhood vasculitis, Kawasaki disease is, again, a vasculitis of predominantly medium-sized vessels. Also, the disease is an autoimmune form of vasculitis that does not bind to antineutrophil cytoplasmic antibodies, unlike other vasculitic diseases associated with them (such as granulomatosis with polyangiitis, microscopic polyangiitis, and Churg-Strauss syndrome). This categorization is considered indispensable for appropriate treatment.

Signs and symptoms

Kawasaki disease often begins with a high, sustained fever that does not respond to conventional treatment (acetaminophen) or ibuprofen. This is the most predominant symptom in Kawasaki disease, a characteristic feature of the acute phase of the course of the disease, usually high (above 39-40 ° C), relapsing and accompanied by excessive irritability. Recently, patients have been reported to have atypical or partial Kawasaki disease; however, this is not observed in 100% of cases. The first day of the fever is considered the first day of illness, and the duration of the fever averages from one to two weeks; in the absence of treatment, it can last from three to four weeks. Prolonged fever is associated with a higher incidence of heart disease. It partially responds to antipyretic drugs and does not stop with the start of antibiotics. However, when appropriate therapy—intravenous immunoglobulin and aspirin—is started, the fever subsides after two days. Bilateral conjunctival inflammation has been reported to be the most common symptom after a fever. Usually it covers the conjunctiva of the eyeball, is not accompanied by suppuration and is painless. It usually begins immediately after the onset of fever during the acute phase of the disease. Anterior uveitis can be detected on examination of the eye with a target lamp. Iritis may also occur. Corneal deposits are other ocular manifestations (detectable with a slit lamp, but usually too small to be visible to the naked eye). Kawasaki disease is manifested by a complex of intraoral symptoms, the most characteristic changes are bright red (erythema), swollen lips (edema) with vertical cracking (fissures) and bleeding. Oropharyngeal mucosa may be bright red, and the tongue may have a typical "crimson tongue" appearance (severe erythema with a predominance of gustatory tubercles). These intraoral symptoms are caused by typical necrotizing microvasculitis with fibrinoid necrosis. Cervical lymph node involvement occurs in 50% to 75% of patients, while other characteristic features are estimated to occur in 90% of patients, but this can sometimes be the dominant symptom. By definition of the diagnostic criterion, at least one affected lymph node ≥ 1.5 cm in diameter must be included. Affected lymph nodes are painless or minimally tender, nonfluctuating, and nonsuppurative; erythema of adjacent skin may be observed. Children with fever and cervical adenitis who do not respond to antibiotics have Kawasaki disease, which is considered part of the differential diagnosis.

Less common symptoms:

Gastrointestinal tract: diarrhea, abdominal pain, vomiting, liver dysfunction, pancreatitis, gallbladder dropsy, parotitis, cholangitis, intestinal intussusception, intestinal pseudo-obstruction, ascites, splenic infarction

Musculoskeletal system: polyarthritis and arthralgia

Cardiovascular system: myocarditis, pericarditis, tachycardia, heart disease

Genitourinary system: urethritis, prostatitis, cystitis, priapism, interstitial nephritis, orchitis, nephrotic syndrome

Central nervous system: lethargy, subcoma, aseptic meningitis, and sensorineural deafness

Respiratory system: shortness of breath, flu-like illness, pleural effusion, atelectasis

Skin: erythema and induration at the site of BCG vaccination, Bo's furrows, and gangrene of the finger

In the acute phase of the course of the disease, changes in the peripheral extremities may include erythema of the palms and soles, which is often affected by acute demarcation and is often accompanied by painful, firm swelling of the dorsum of the hands and feet. This is the reason why affected children often refuse to hold objects in their hands and bear weight on their feet. Later, during the convalescent or subacute phase, peeling of the fingers and toes usually begins in the periungual area within two to three weeks of the onset of the fever and may spread to the palms and soles. Approximately 11% of affected children may have skin peeling for many years. One to two months after the onset of a fever, deep transverse grooves on the nails (Beau's furrows) may develop, sometimes the nails come off. The most common skin symptom is a diffuse maculopapular erythematous rash, which is fairly nonspecific. The rash changes over time and is characteristically located on the trunk; in the future, it can spread to the face, limbs and perineum. Many other forms of skin lesions have been reported; they may include scarlatiniform, papular, urticaria-like lesions, erythema multiforme, and purpuric lesions; even micropustules have been reported. They may be polymorphic, non-pruritic, and usually present before the fifth day of the heat. However, they are never bullous or vesicular. In the acute stage of Kawasaki disease, systemic inflammatory changes are seen in many organs. Joint pain (arthralgia) and swelling, often symmetrical, and arthritis may also occur. Myocarditis, diarrhea, pericarditis, valvutitis, aseptic meningitis, pneumonia, lymphadenitis, and hepatitis can and do manifest themselves through the presence of inflammatory cells in exposed tissues. If left untreated, some symptoms will eventually improve, but coronary artery aneurysms do not improve, leading to a significant risk of death and disability due to myocardial infarction. If treated quickly, this risk can be avoided in most cases and the course of the disease will be shorter. Other non-specific symptoms reported include cough, runny nose, sputum, vomiting, headache, and seizures.

The course of the disease can be divided into three clinical phases:

Clinical manifestations differ between adults and children, with cervical adults being more affected by lymph nodes (93% of adults versus 15% of children), hepatitis (65% versus 10%), and arthralgia (61% versus 24% of children). 38%). Some patients have atypical presentations and may not have the usual symptoms. This is seen in particular in infants; these patients are at increased risk for aneurysms of the cardiac arteries.

Complications

cardiac

Cardiac complications represent the most important aspect of Kawasaki disease. It is the leading cause of childhood acquired cardiovascular disease in the United States and Japan. In developed countries, it is being replaced by acute rheumatic fever as the most common cause of childhood acquired cardiovascular disease. Coronary artery aneurysms occur as a complication of vasculitis in 20-25% of untreated children. They are first detected after an average of 10 days of illness, and the peak incidence of dilatation or aneurysm of the coronary arteries is observed within four weeks after the onset of the disease. Aneurysms are classified into small (internal diameter of the vascular wall<5 мм), средние (диаметр в диапазоне 5–8 мм) и крупные (диаметр > 8 mm). Saccular and fusiform aneurysms usually develop between 18 and 25 days after onset. Even when treated with high-dose IVIG during the first 10 days of illness, 5% of children with Kawasaki disease develop at least temporary dilatation of the coronary arteries, and 1% develop large aneurysms. Death can occur either due to myocardial infarction due to the formation of blood clots in the aneurysm of the coronary artery, or due to the rupture of a large aneurysm of the coronary artery. The lethal outcome is most often observed from the second to the 12th week after the onset of the disease. Many risk factors preceding coronary artery aneurysms can be identified, including persistent fever after IVIG therapy, low hemoglobin, low albumin, high white blood cell count, high stab neutrophil count, high C-reactive protein, male gender, and age less than one year. Damage to the coronary arteries due to Kawasaki disease changes dynamically over time. Resorption after one to two weeks after the onset of the disease is observed in half of the vessels with aneurysms. The narrowing of a coronary artery that occurs as a result of the healing process of the vessel wall often leads to significant obstruction of the blood vessel and results in the heart not receiving enough blood and oxygen. This can eventually lead to the death of heart muscle tissue (myocardial infarction). Myocardial infarction caused by thrombotic occlusion in an aneurysmal, stenotic, or aneurysmal and stenotic coronary artery is the leading cause of death from Kawasaki disease. The highest risk of myocardial infarction occurs in the first year of the course of the disease. Myocardial infarction in children presents with symptoms that differ from adults. The main symptoms are shock, anxiety, vomiting, and abdominal pain; chest pain is common in older children. Most of these children have an exacerbation during sleep or rest, with about one-third of the exacerbations being asymptomatic. Valve insufficiency, particularly mitral or tricuspid valve insufficiency, is often seen in the acute course of Kawasaki disease due to valvular inflammation or myocardial inflammation-induced myocardial dysfunction, regardless of the involvement of the coronary arteries. These lesions mostly disappear with the cessation of the acute phase of the disease, but a small group of lesions persist and progress. Late-onset aortic or mitral regurgitation, caused by thinning or deformity of the fibrous valves, is also seen, ranging from months to years after the onset of Kawasaki disease. Some of these damages necessitate valve replacement.

Other

Other complications of Kawasaki disease have been described, such as aneurysms of other arteries: aortic aneurysm, with a large number of cases including abdominal aorta, axillary aneurysm, aneurysm of the brachycephalic artery, aneurysms of the iliac and femoral arteries, and aneurysm of the renal artery. Other vascular complications may occur, such as increased wall thinning and reduced distension of the carotid arteries, aorta, and brachioradialis. This change in vascular tone occurs due to endothelial dysfunction. In addition, children with Kawasaki disease, with or without coronary artery complications, may have a more adverse cardiovascular risk profile such as high blood pressure, obesity, and an abnormal serum lipid profile. Gastrointestinal complications of Kawasaki disease are similar to those seen in Henoch-Schonlein purpura, such as ileus, colonic edema, intestinal ischemia, pseudo-ileus, and acute abdomen. Eye changes associated with the disease have been described since the 1980s and include uveitis, iridocyclitis, conjunctival hemorrhage, optic neuritis, amaurosis, and occlusion of the optic artery. Necrotizing vasculitis progressing to peripheral gangrene may also be found. Neurological complications due to damage to the central nervous system are reported much more frequently. Neurological complications found include meningoencephalitis, subdural effusion, cerebral hypoperfusion, cerebral ischemia and infarction, cerebellar infarction manifesting through paroxysms, chorea, hemiplegia, confusion, lethargy and coma, or even cerebral infarction without neurological manifestations. Other neurological complications resulting from cranial nerve involvement include ataxia, prosopoplegia, and sensorineural hair loss. Behavioral changes thought to be caused by local cerebral hypoperfusion may include attention deficits, cognition deficits, emotional disturbances (emotional instability, fear of the dark, and nightmares), and internalization problems (anxiety, depressive or aggressive behavior).

The reasons

Since the cause(s) of Kawasaki disease remain unknown, the disease is more accurately described as Kawasaki syndrome. As with all autoimmune diseases, its cause is thought to be an interaction between genetic and environmental factors, possibly including infection. The specific cause is not known, but current theories focus primarily on immunological causes. Evidence mostly points to an infectious etiology, but debate continues as to whether the cause lies in a common antigenic substance or a superantigen. Boston Children's Hospital researchers reported that “some studies have found an association between the occurrence of Kawasaki disease and recent exposure to carpet cleaning or living near stagnant water; however, cause and effect have not been established." Other data show an obvious relationship between Kawasaki disease and tropospheric wind patterns; winds blowing from Central Asia have been linked to cases of Kawasaki disease in Japan, Hawaii and San Diego. This association with tropospheric winds is modulated along the seasonal and interannual time scale of El Niño, the southern oscillation phenomenon, further suggesting that the agent responsible for the disease is a wind-borne pathogen. Activities are represented by the process of identifying a suspicious pathogen in air purification facilities installed at a height above Japan. An association has been established with a single nucleotide polymorphism in the ITPKC gene, which encodes an enzyme that negatively regulates T-cell activation. Regardless of where they live, Japanese children are more likely to develop the disease than other children, suggesting a genetic predisposition. The HLA-B51 serotype has been associated with endemic cases of the disease.

Diagnostics

Kawasaki disease can only be diagnosed clinically (that is, through clinical signs and symptoms). There are no specific laboratory tests for this disease. Diagnosis is difficult, especially early in the course of the disease, and often children are not diagnosed until they have been seen by several healthcare professionals. Many other serious illnesses can cause similar symptoms and should be considered in the differential diagnosis, including scarlet fever, toxic shock, juvenile idiopathic arthritis, and childhood mercury poisoning (acrodynia infancy). Usually five days of fever and four of the five diagnostic criteria are enough to make a diagnosis.

Criteria include:

Erythema of the lips or mouth, or cracked lips

Rash on the body

Swelling or erythema of hands and feet

Red eyes (conjunctival infection)

Swollen lymph node in the neck, at least 15 mm in size

Many children, especially infants, end up being diagnosed with Kawasaki disease without demonstrating all of the above criteria. In fact, many experts now recommend treatment for Kawasaki disease if only three days of fever have passed and at least three diagnostic criteria are present, especially if other tests have found abnormalities consistent with Kawasaki disease. In addition, the diagnosis can be made accurately by detecting coronary artery aneurysms in an appropriate clinical setting.

Interventions

A physical examination will show many of the features listed above.

Blood tests:

Complete blood count may detect normocytic anemia and eventually thrombocytosis.

The erythrocyte sedimentation rate will be increased.

C-reactive protein will be elevated.

Liver function tests may show inflammation of the liver and low serum albumin levels.

Other agreed-upon tests include:

An electrocardiogram may show the presence of ventricular dysfunction or, rarely, an arrhythmia due to myocarditis.

An echocardiogram may show subtle changes in the coronary arteries or, later, true aneurysms.

An ultrasound or CT scan may show hydrops (enlargement) of the gallbladder.

Urinalysis may show levels of white blood cells and protein in the urine (pyuria and proteinuria) without obvious bacterial overgrowth.

Lumbar puncture may show the presence of aseptic meningitis.

X-ray angiography has historically been used to detect coronary artery aneurysms and remains the gold standard for their detection, but is rarely used today if coronary artery aneurysms have already been detected on electrocardiography.

Temporal artery biopsy

Treatment

Children with Kawasaki disease should be hospitalized and monitored by a doctor who is experienced with the disease. In an academic medical center, nursing is often divided between pediatric cardiology, pediatric rheumatology, and pediatric infectious disease specialists (although the specific causative agent of the infection has not yet been identified). Treatment should begin immediately after diagnosis to avoid damage to the coronary arteries. Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease and is given in high doses, with significant improvements usually seen within 24 hours. If the fever does not respond to treatment, an additional dose may be used. In rare cases, a child may receive a third dose. IVIG alone is most useful during the first seven days after the onset of a fever in preventing coronary artery aneurysms. Therapy with salicylic acid, in particular aspirin, remains an important component of treatment (although questioned by some), but salicylates alone are not as effective as IVIG. Aspirin therapy is started at high doses and continued until the fever subsides, and then continued at low doses when the patient returns home, usually for two months, to prevent blood clots. With the exception of Kawasaki disease and a few other indications, aspirin is generally not recommended for children due to its association with Reye's syndrome. Because children with Kawasaki disease take aspirin for up to several months, varicella and flu vaccinations are required, as these infections most commonly cause Reye's syndrome. Corticosteroids are also used, especially when other treatments have failed or symptoms have returned, but in a randomized controlled trial, adding a corticosteroid to immunoglobulin and aspirin did not improve outcomes. In addition, the use of corticosteroids in the setting of Kawasaki disease is associated with an increased risk of coronary artery aneurysm, so the use of corticosteroids is generally contraindicated in these circumstances. In cases of difficult-to-treat IVIG Kawasaki disease, cyclophosphamide and plasmapheresis have been investigated as possible treatments with variable results. An IL-1 receptor antagonist (anakinra) may prevent coronary injury in a mouse model of knee disarticulation. This prevention is found even with a three-day delay in treatment in mice. There are ways to treat iritis and other eye symptoms. Another method of treatment may include the use of infliximab. Infliximab works by binding to tumor necrosis factor alpha.

Forecast

With early treatment, rapid resolution of acute symptoms can be expected, and the risk of coronary artery aneurysms is greatly reduced. If left untreated, the acute symptoms of Kawasaki disease are self-limiting (i.e., the patient eventually recovers), but the risk of coronary artery involvement is much higher. Overall, about 2% of patients die from complications of coronary vasculitis. Patients with Kawasaki disease should have an echocardiogram initially every few weeks and then every one or two years to check for progression of cardiac complications. Laboratory evidence of increased inflammation, combined with demographic characteristics (male, less than six months of age or greater than eight years of age) and inadequate response to IVIG therapy, create a profile of patients at increased risk for Kawasaki disease. It is likely that an aneurysm is largely characterized by its original size, with small aneurysms being more likely to regress. Other factors positively associated with aneurysm regression include age <1 year at onset of Kawasaki disease, fusiform rather than saccular aneurysm morphology, and distal coronal aneurysm location. An increased rate of progression to stenosis is seen among those who develop large aneurysms. The worst prognosis awaits children with large aneurysms. This serious outcome may require further treatment such as percutaneous transluminal angioplasty, coronary artery stenting, implantation of a heart-lung machine, and even a heart transplant. A return of symptoms may occur shortly after initial IVIG treatment. This usually requires readmission and treatment. IVIG treatment can cause allergic and non-allergic acute reactions, aseptic meningitis, hypervolemia and, in rare cases, other serious reactions. In general, life-threatening complications resulting from treatment for Kawasaki disease are relatively rare, especially when compared with the risks associated with no treatment. In addition, there is evidence that Kawasaki disease alters fat metabolism, which persists after clinical regression of the disease.

Epidemiology

Boys are more susceptible to Kawasaki disease than girls, with people of Asian ethnicity, in particular those of Japan and Korea, being the most predisposed, as well as people of Afro-Caribbean ethnicity. The disease was rare in Caucasians until the last few decades, with prevalence varying from country to country. Today, Kawasaki disease is the most commonly diagnosed pediatric vasculitis in the world. By far the highest incidence of Kawasaki disease is in Japan, with a more recent study estimating an incidence of 218.6 per 100,000 children under the age of five (about one in 450 children). According to this incidence rate, more than one in 150 children in Japan will develop Kawasaki disease during their lifetime. However, the incidence in the United States is on the rise. Kawasaki disease is predominantly a disease of young children, with 80% of patients under five years of age. Approximately 2000-4000 cases are established in the US each year (between 9 and 19 per 100,000 children under five). In the UK, incidence estimates vary due to the rarity of Kawasaki disease. However, the disease is said to affect less than one in 25,000 people. The incidence of the disease doubled from 1991 to 2000, with four cases per 100,000 children in 1991 compared to eight cases per 100,000 in 2000.

Story

The disease was first reported by Tomisaku Kawasaki in a four-year-old child with a rash and fever at a Red Cross hospital in Tokyo in January 1961, and later published a report of 50 similar cases. Later, Kawasaki and colleagues became convinced of a definite cardiac involvement when they examined and reported 23 cases, of which 11 (48%) patients showed abnormalities detected by electrocardiogram. In 1974, the first description of this disease was published in the English literature. In 1976, Melish et al. described a similar condition in 16 children in Hawaii. Melisch and Kawasaki independently developed similar diagnostic criteria for the disease that are used today to diagnose classic Kawasaki disease. The question was raised whether the disease appeared during the period from 1960 to 1970, but the later preserved heart of a seven-year-old boy who died in 1870 was examined, as a result of which three aneurysms of the coronary arteries with thrombi were found, as well as pathological changes, related Kawasaki disease. Kawasaki disease is currently being diagnosed worldwide. In the US and other developed countries, it is being replaced by acute rheumatic fever as the most common cause of childhood acquired cardiovascular disease.

:Tags

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Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. pp. 1232–4. ISBN 1-4160-2999-0.

Okano M, Luka J, Thiele GM, Sakiyama Y, Matsumoto S, Purtilo DT (October 1989). "Human herpesvirus 6 infection and Kawasaki disease". Journal of Clinical Microbiology 27(10): 2379–80. PMC 267029. PMID 2555393.

Kim DS (December 2006). Kawasaki disease. Yonsei Medical Journal 47(6): 759–72. doi:10.3349/ymj.2006.47.6.759. PMC 2687814. PMID 17191303.

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Kawasaki disease is a generalized vasculitis of unknown etiology, also called mucocutaneous lymph node syndrome, or periarteritis nodosa of childhood. In developed countries, Kawasaki disease has overtaken acute rheumatic fever as the cause of acquired heart disease.

In the occurrence of the disease, hereditary factors matter; the infectious nature of the disease is assumed.

The estimated prevalence in the United States is 10 per 100,000 children under 5 (non-Asians) and 44 per 100,000 (Asians). Mortality is about 1%. Without treatment, aneurysms of the coronary arteries develop in 25% of children. When treated with gamma globulin and when it starts earlier than the 10th day, the incidence of aneurysms of the coronary arteries is reduced to 5-10%. 80% of patients diagnosed with Kawasaki disease are children under 4 years of age. Up to 3 years, the disease rarely develops.

Pathogenesis

The central role in the genesis of this vasculitis is attributed to antibodies to endothelial cells and viruses. In the affected vessels, inflammation of the media develops with necrosis of smooth muscle cells. Arterial aneurysms may form due to delamination of the elastic layer. After 4-8 weeks, fibrosis of the vessel wall develops, proliferation and thickening of the intima; acute general symptoms of the disease develop. In the affected vessels, stenosis develops, thrombosis and occlusion are possible. So, with Kawasaki disease, it can develop due to thrombosis or rupture of an aneurysm of the coronary artery. Arteries of medium diameter are affected: renal, periovarian, splanchnic, axillary, etc., as well as veins, capillaries, arterioles and larger arteries.

Clinic

In young children, after an initial 3-4 week phase with a temperature of 38-40°C, microaneurysms form in the coronary arteries. Fever usually above 39.9 ° C lasts at least 5 days, is intermittent. Without treatment, it may be delayed for 1-2 weeks, may not respond to antipyretics, but disappears 1-2 days after the start of treatment with intravenous gamma globulin. Along with this, nonspecific skin exanthema, general severe signs of the disease, conjunctivitis, chapped lips, crimson tongue and painful swelling of the lymph nodes appear. Plantar and palmar erythema is characteristic, accompanied by edema and subsequent desquamation (see table).

Diagnostics

In the analyzes, leukocytosis due to granulocytosis is noted, there may be anemia and thrombocytosis at 2-3 weeks (up to 700 * 10 9 / l). Increased ESR, C-reactive protein, α1-antitrypsin. Complement remains normal. Transaminases may be slightly elevated (in 40% of patients).

In the general analysis of urine, pyuria, proteinutria may be noted.

Mycardial infarction is diagnosed by cardiospecific enzymes and ECG. The ECG can also show signs of myocarditis, myocardial infarction.

Chest x-ray may show cardiomegaly. The state of the heart is monitored by echocardiography. It is possible to perform CT angiography and MR angiography of the heart.

Kawasaki syndrome is often a diagnosis of exclusion; differential diagnosis is carried out with scarlet fever, staphylococcal skin syndrome, Steven-Jones syndrome, drug reactions, JRA, measles, mercury poisoning, viral exanthems.

Treatment

The main goal of therapy for Kawasaki disease is to prevent the development of aneurysms of the coronary arteries, which will reduce mortality. The basis of therapy is intravenous gamma globulin (IVIG) in a full dose - 2 g / kg for 10-12 hours. In addition, aspirin and possibly corticosteroid hormones are usually prescribed.

The traditional treatment for Kawasaki disease is aspirin. However, according to some studies, aspirin in high and medium doses (up to 100 mg / kg daily in 2-4 doses for a period of fever, after a decrease in temperature - 3-5 mg / kg / day for 3 months) even with early onset therapy did not increase the effectiveness of intravenous gamma globulin, i.e. did not affect the duration of fever and the incidence of aneurysms of the coronary arteries. Therefore, the expediency of prescribing salicylates in Kawasaki disease remains controversial.

The use of glucocorticosteroids is also insufficiently studied. According to one randomized Japanese study, corticosteroids in addition to intravenous immunoglobulin did not reduce the incidence of coronary artery aneurysms, did not significantly affect the duration of fever, C-reactive protein levels. In another US randomized trial, a single dose of methylprednisolone (30 mg/kg) in addition to conventional therapy resulted in a significant decrease in ESR one week after the start of treatment, but did not affect the incidence of coronary artery anomalies.

During treatment, it is important to conduct dynamic cardiac monitoring (see above). Once the fever resolves, the development of cardiac involvement is unlikely. It is important to repeat cardiac echoscopic examination approximately one month after the onset of fever. A rather rare complication is mitral valve endocarditis requiring valve replacement. Patients with large coronary artery aneurysms are subsequently given antiplatelet therapy (usually aspirin). The frequency of recurrence of the disease is from 1 to 3%.

Kawasaki disease in children is an inflammatory lesion of arteries of any diameter, accompanied by an increase in body temperature, redness of the conjunctiva, an increase in cervical lymph nodes, and pathological changes in the mucous membrane of the pharynx and oral cavity.

The danger of the disease is that the vessels of the heart, the aorta and large veins can be affected. Therefore, early diagnosis is required, which allows subsequent effective treatment and prevention of serious complications.

The disease most often occurs in childhood. In this case, the peak occurs at 2 years of age, but the increased risk persists up to 5 years. According to statistics, this is 85% of cases of diagnosed Kawasaki disease in pediatric practice.

The immediate causes of Kawasaki disease are not known. The following factors are considered as predisposing factors:

• adenovirus infection
• scarlet fever.

The main damaging mechanism in these infections is immune:

• increased levels of cytokines in the blood that support the inflammatory response
• increased activity of macrophages and monocytes
• antibodies are formed against antigens localized in the vascular wall.

These processes lead to damage to the arterial wall. It becomes thinner, and aneurysms are formed - expansions that can rupture. Blood clots form in the veins, which pose an additional danger, because. with the blood flow can get into any organ (heart, lungs, kidneys, etc.), dramatically disrupting its functions.

The highest prevalence of the disease was noted among the Japanese, the lowest - among the inhabitants of South America. Scientists cannot yet explain such a geographical spread.

Symptoms

Main symptoms of kawasaki disease in children include:

1. fever
2. changes in the hands and feet
3. skin rash
4. lymphadenopathy
5. eye redness
6. changes in the oral and pharyngeal mucosa.

With this disease, the temperature rises to 38-40 ° C and lasts up to 5 days, and sometimes longer (up to a month). An important diagnostic feature is ineffectiveness of antipyretic and antibacterial agents to relieve fever. The temperature is reduced only by the introduction of immunoglobulin.

2-3 days after the onset of fever, the eyes turn red due to the injection of conjunctival vessels. Redness persists for 10-15 days if the child is not given specific treatment. Then it gradually disappears, leaving no ulcers on the cornea, unlike infectious diseases of the eyes.

• cracks
• dryness
• diffuse redness
• crimson tongue.

Therefore, the child's face has a characteristic appearance - red eyes and red lips. The appearance of such a sign against the background of elevated temperature should alert parents regarding Kawasaki disease. For an accurate diagnosis, you need to contact a pediatrician.

Cervical lymphadenopathy is detected in half of the cases and more often (up to 70%). Lymph nodes are painless, enlarged on one or both sides. One of them reaches a significant size - up to 1.5 cm or more. Parents can palpate them at home.

The feet and hands are affected in 90% of children with Kawasaki disease. It manifests itself in various ways:

• diffuse thickening and swelling
• redness of palmar and plantar surfaces
• after 2 weeks, peeling appears, starting at the fingertips and spreading towards the body.

Skin rash appears on the limbs and trunk. The eruptions vary in form, resembling scarlet fever and other diseases. However, skin elements are never represented by vesicles. Sometimes children may develop small, purulent pimples on their ankles, knees, and elbows.

Additional examination

Laboratory tests for Kawasaki disease in children do not have specific signs. These patients may experience:

1. increased white blood cell count
2. the appearance of "young" forms (not having time to finally differentiate) leukocytes
3. acceleration of ESR, which is combined with fever
4. an increase in C-reactive protein that persists for 1-1.5 months
5. thrombocytosis (up to 800 thousand), reaching a maximum a week after the onset of the disease
6. increased blood clotting
7. in the urine - a large number of leukocytes in the absence of bacteria.

Instrumental studies help to identify the most common complications:

• conduction disturbances on ECG
• coronary angiography (X-ray examination of the arteries of the heart) detects aneurysms and narrowing of the vascular lumen
• radiography of the lung tissue. In 10% of cases, small nodules, reticulation, and atelectasis (areas of collapsed lungs) can be detected.

Accurate Diagnosis

In 1984, Japanese scientists developed clear criteria for diagnosing Kawasaki disease, and in 2006 they were supplemented taking into account modern realities.

This diagnosis is made if there is a mandatory sign in combination with one of the additional ones. A fever persisting for at least 5 days is mandatory. Additional are:

• bilateral reddening of the conjunctiva
• skin changes on the limbs or on the perineum
• polymorphic (different in shape) rashes on the skin
• enlarged cervical lymph nodes
• redness of the tongue, oral mucosa, lips and pharynx.

Complications

The most serious complications of Kawasaki disease are cardiovascular:

1. ischemic disease
2. myocardial infarction
3. pericarditis
4. myocarditis
5. endocarditis
6. sudden death.

But there may be others, also due to immune inflammation:

• arthritis
• cholecystitis
• meningitis
• endarteritis
• aneurysms of the renal arteries.

Treatment

The main treatment for Kawasaki disease is the administration of immunoglobulin intravenously. In the acute phase, it is used once. If symptoms persist, repeat administration. In parallel with immunoglobulins, acetylsalicylic acid is prescribed in high doses. It prevents the formation of blood clots in the vessels, which pose an immediate danger to the child. After 10 days, the dose of acetylsalicylic acid is gradually reduced and continued to be taken for a month with the obligatory control of the coagulation system (normally there should not be a state of hypercoagulability - a tendency to thrombosis).

AT in cases where immunoglobulins are ineffective, the use of corticosteroids.

They suppress the severity of immune inflammation. At the first stage, pulse therapy is used (high doses of hormones), and subsequently gradually decreasing doses. This approach will avoid the development of side effects of corticosteroid therapy.

Immunosuppressive treatment allows to achieve regression of coronary aneurysms (within 6 months - one and a half years). However, if their size is greater than 8 mm, then they do not disappear, so surgery is required. Otherwise, the child increases the risk of thrombosis of the heart vessels and myocardial infarction. This explains the possibility of death in 0.1-0.2% of cases. Timely treatment is associated with a favorable prognosis for a person throughout his life.

How to identify and treat Kawasaki disease in a child updated: May 6, 2016 by: admin

Kawasaki is a disease that usually occurs in children from one and a half to 5 years old. Most often, the disease begins between 1.5 and 2 years. In addition, Kawasaki is a disease that affects boys more than girls (1.5:1).

How can this disease be described? Kawasaki syndrome is characterized by inflammation of the walls of blood vessels, leading to dilatation of mainly the coronary arteries. There is also fever, vasculitis and changes in the lymph nodes, mucous membranes of the skin.

Story

This disease was first described by the Japanese pediatrician T. Kawasaki in 1967. He introduced it as a new childhood disease - mucocutaneous lymphnodular syndrome. In total, he observed 50 cases of the disease. All children had enlarged lymph nodes, cracks on the lips, exanthema, swelling of the soles and palms, hyperemia. At first, this disease was considered easily curable, but after several deaths, it was found that patients had serious heart damage. Subsequently, the whole world learned about the discovery of the scientist, and the disease was named after him.

The reasons

Currently, medicine does not know the causes of Kawasaki disease. However, there is evidence that Kawasaki is a disease that develops due to an infectious agent in genetically predisposed patients.

This disease is 10 times more common in Japan than in America, and almost 30 times more common than in Britain and Australia. It was noted that in winter and spring they get sick more often.

Kawasaki disease symptoms

The disease is characterized by the following symptoms:

Fever that persists for more than 5 days.

Cracks appear on the lips, it often happens that they bleed. There are foci of erythema.

There are hemorrhages in the oral mucosa.

Kawasaki is a disease in children, which is characterized by a bright color of the tongue.

Nasal congestion and hyperemia of the pharynx are observed.

The body temperature rises.

Conjunctivitis appears due to increased filling of blood vessels.

The skin on the fingertips begins to peel off (at 2-3 weeks of the course of the disease).

There is exanthema on the skin of the body, erythema on the feet and palms.

Dense swelling appears on the hands and feet. This usually occurs 3-5 days after the onset of the disease.

The child quickly gets tired and becomes very irritable.

Kawasaki syndrome is also characterized by an increased heart rate. Since the heart is damaged, the heart rhythm may be disturbed.

The lymph nodes usually become larger in the neck.

Over time, various complications from many systems and organs can be observed. Perhaps the development of myocarditis, myocardial infarction from the side of the cardiovascular system. With the disease, there is a possibility of rupture of the aneurysm, while blood enters the pericardial cavity, thereby developing hemopericardium. Many rare diseases, including Kawasaki disease, cause damage to the heart valves. They can develop an inflammatory process, which is called valvulitis. If the gallbladder is affected, then dropsy is formed, meninges - aseptic meningitis, joints - arthritis, ear cavities - otitis media. With blockage of large arteries that are located in the limbs, gangrene is formed.

Diagnosis of the disease

The patient is diagnosed on the basis of clinical signs and, of course, diagnostic examinations are mandatory.

Blood test - thrombocytosis, anemia, transaminases, elevated levels of C-reactive protein, ESR, antitrypsin are detected;

Urinalysis - there is the presence of pus and protein in the urine;

Electrocardiography - used to make an early diagnosis of myocardial infarction;

X-ray of the chest organs - allows you to detect changes in the boundaries of the heart;

Magnetic resonance and computed angiography - are necessary in order to assess the patency of the coronary arteries;

Echocardiography - makes it possible to determine whether there are abnormalities in the work of the heart.

Difficulty in making a diagnosis

Rare diseases are usually not so common among doctors. They only know about them from books. Therefore, it is more difficult to make a diagnosis, such as Kawasaki syndrome, in young children than in older children. However, most often it is in young patients after the disease that complications occur in the heart. A child suffering from this disease is usually irritable and inconsolable (this may be due to aseptic meningitis), but such signs can also be with other infections, such as measles.

Another sign is hardening and redness of the BCG injection site due to cross-reactivity between T cells and heat shock proteins.

Changes in the oral cavity, as well as peripheral changes and a rash, can also be with scarlet fever. Although enlarged lymph nodes and conjunctivitis are not observed with it.

Kawasaki is a disease that can also be confused with scalded skin syndrome, rubella, roseola infantum, Epstein-Barr virus, infectious mononucleosis, influenza A, toxic shock syndrome, adenovirus infection, Stevenson-Johnson syndrome, systemic juvenile rheumatoid arthritis.

Stages of the disease

1. Acute febrile. It lasts the first two weeks, the main signs are fever and symptoms of acute inflammation.

2. Subacute. It lasts from 2 to 3 weeks, is characterized by an increase in the level of platelets, the appearance of aneurysms is possible.

3. Recovery. Usually at 6-8 weeks after the onset of the disease, while all the symptoms of the disease disappear, the stage continues to normal ESR and the lesions of blood vessels decrease or resolve.

Kawasaki disease, like all febrile diseases, begins acutely with a sharp increase in body temperature up to 40 ° C. Also characteristic is the strong excitability of patients. Patients suffer from fever, often suffer from pain in the abdomen and small joints. If no measures are taken, then the fever lasts from 1 to 2 weeks, but sometimes up to 36 days.

Treatment

Usually consists of two stages. At the first stage, aspirin is used or intravenous immunoglobulin is administered. These drugs are needed to prevent the formation of aneurysms in the coronary arteries. The best effect of treatment is achieved if you start it in the first 10 days from the onset of the acute stage of the disease.

It is known that after undergoing this therapy, most children are cured of Kawasaki syndrome. However, recent studies show that these patients may develop coronary artery abnormalities over time. That is why people who have suffered this terrible disease need to be examined at least once every 5 years with the help of an echocardiograph and be observed by a cardiologist.

At the second stage, the treatment of aneurysms that have appeared in the coronary artery is organized. If they are detected, an additional course of aspirin is prescribed, it is also necessary to periodically undergo an examination (echocardiography and possibly angiography of the coronary vessels). In the case when the aneurysm of the artery is large, then it is possible to prescribe an additional anticoagulant (clopidogrel or warfarin).

If coronary artery stenosis has developed, catheterization, rotational ablation and artery bypass are prescribed.

Impact on the heart

Kawasaki is a disease that causes heart failure in children, although not in all cases. The heart is involved in the pathological process in the first few days of illness or after the crisis. The acute form of the disease is characterized by the development of an inflammatory process in the heart muscle (myocardium). However, in most cases, after this there are no serious consequences, but sometimes it can be an incentive for the development of congestive heart failure. The heart muscle is weakened and cannot function normally. This causes the accumulation of fluid in the tissues and the formation of edema.

Kawasaki disease in adults

Does the ailment remind of itself over time? It is known that many people who have had rare diseases recover, and there is no trace of the disease. In the case of Kawasaki syndrome, one in five people experiences severe consequences from the heart and blood vessels that feed the heart muscle. In this case, the walls of the vessels lose their elasticity and elasticity, and aneurysms are also observed (swelling of some areas). This leads to early onset of atherosclerosis or calcification. Sometimes all this leads to the formation of blood clots, malnutrition of the heart muscle occurs and, in the end, myocardial infarction occurs.

Usually, over time, the aneurysms that develop with the disease become smaller. It has been found that the younger the age of a person at the time of the appearance of neoplasms, the more likely it is that they will finally disappear over time. Aneurysms that persist in adults can cause stenosis, blockage, thrombosis, which can lead to a heart attack. Therefore, examinations of such patients become very important in order to exclude the possibility of adverse symptoms in the future.