Mucocutaneous Lymph Node Syndrome
Lymph Nodes
Candidiasis, Chronic Mucocutaneous
Leishmaniasis, Mucocutaneous
Coronary Aneurysm
Immunoglobulins, Intravenous
Encyclopedias as Topic
Aneurysm
Intracranial Aneurysm
Polyclonal expansion of TCRBV2- and TCRBV6-bearing T cells in patients with Kawasaki disease. (1/526)
We examined T-cell receptor (TCR) usage, cytokine production and antibody responses to superantigens in patients with Kawasaki disease (KD) to facilitate a better understanding of the immunopathogenesis of KD. The mean percentage of VB2- or VB6. 5-bearing T cells in peripheral blood mononuclear cells (PBMC) of patients with acute-phase KD was significantly higher than that of patients in the convalescent phase of KD or in healthy donors. Expansion of VB2- or VB6.5-bearing T cells was polyclonal because DNA sequences in the complementarity determining region 3 of VB2- and VB6.5-positive cDNA clones were all different from each other. The plasma levels of interleukin (IL)-1beta, IL-2, IL-6, IL-8, IL-10, interferon-gamma (IFN-gamma), tumour necrosis factor-alpha (TNF-alpha) and granulocyte colony-stimulating factor (G-CSF) were elevated in the acute phase of KD. We previously reported that streptococcal pyrogenic exotoxin C (SPEC) was a potent stimulator of VB2- and VB6.5-positive T cells and, furthermore, serum levels of anti-SPEC antibodies were significantly higher in patients with acute and convalescent KD than in age-matched controls. The results of the present study, together with those of our previous report, suggest that SPEC induces activation and polyclonal expansion of VB2- and VB6.5-positive T cells, and that SPEC-induced activation of T cells may lead to the pathogenesis of KD. (+info)Dramatic decrease of circulating levels of monocyte chemoattractant protein-1 in Kawasaki disease after gamma globulin treatment. (2/526)
Kawasaki disease (KD) is a systemic vasculitis preferentially affecting coronary arteries. Extensive monocytes/macrophages infiltrate in the vascular lesions, implying the involvement of a chemotactic cytokine in their recruitment. We investigated the role of monocyte chemoattractant protein-1 (MCP-1, also termed monocyte chemotactic and activating factor) in KD. In the immunohistochemical studies using the cardiac tissues of patients with fatal KD, MCP-1 but not interleukin (IL) -8 or macrophage inflammatory protein-1alpha was localized at the extracellular matrix associated with mononuclear cellular infiltration. The sites of MCP-1 expression correlated with the distribution of the acute inflammation, including early coronary vasculitis. In prospectively studied patients with KD, circulating levels of MCP-1, IL-8, tumor necrosis factor alpha (TNF-alpha), and IL-1alpha were elevated in 73, 77, 57, and 0% of samples before gamma globulin (GG) treatment (400 mg/kg x 5 days = total 2 g/kg), respectively, compared with respective control values. GG treatment correlated with a rapid decrease in the circulating levels of MCP-1 (P = 0.001) but not IL-8 (P = 0.19) or TNF-alpha (P = 0.33). In the sensitive Western blotting, MCP-1 bound to GG. Furthermore, GG inhibited the MCP-1-induced Ca2+ influx in a human monocytic cell line in vitro. These findings suggest a role of MCP-1 in KD, and indicate that GG treatment may block MCP-1 activity, thus alleviating KD vasculitis. (+info)Decreased interferon-gamma (IFN-gamma)-producing T cells in patients with acute Kawasaki disease. (3/526)
Kawasaki disease (KD) is an acute febrile illness of early childhood, in which the activation of monocytes/macrophages plays a central role in the development of vasculitis during the acute stage of disease. In this study we investigated peripheral blood T cells of 10 patients with KD, focusing on the Th1 and Th2 imbalance, using intracellular cytokine staining and analysis of the cytokine-producing T cells by flow cytometry. We observed a decrease in the numbers of IFN-gamma-producing, but not IL-4-producing, CD3+ T cells, during the acute stage. Our results suggest that there is an imbalance of Th1 and Th2 subsets during the acute stage of KD. (+info)Anti-human cardiac myosin autoantibodies in Kawasaki syndrome. (4/526)
Kawasaki syndrome (KS) is the major cause of acquired heart disease in children. Although acute myocarditis is observed in most patients with KS, its pathogenesis is unknown. Because antimyosin autoantibodies are present in autoimmune myocarditis and rheumatic carditis, the purpose of the current study was to determine whether anticardiac myosin Abs might be present during the acute stage of KS. Sera from KS patients as well as age-matched febrile controls and normal adults were compared for reactivity with human cardiac myosin in ELISAs and Western blot assays. A total of 5 of 13 KS sera, as compared with 5 of 8 acute rheumatic fever sera, contained Ab titers to human cardiac myosin that were significantly higher than those found in control sera. Both cardiac and skeletal myosins were recognized in the ELISA by KS sera, although stronger reactivity was observed to human cardiac myosin. Only IgM antimyosin Abs from KS sera were significantly elevated relative to control sera. KS sera containing antimyosin Abs recognized synthetic peptides from the light meromyosin region of the human cardiac myosin molecule and had a different pattern of reactivity than acute rheumatic fever sera, further supporting the association of antimyosin Ab with KS. These Abs may contribute to the pathogenesis of acute myocarditis found in patients with KS. (+info)Regulation of the expression of Fc gamma receptor on circulating neutrophils and monocytes in Kawasaki disease. (5/526)
To investigate the regulation of Fc gamma receptor (Fc gamma R) expression on circulating phagocytes in Kawasaki disease (KD), we analysed the expressions of Fc gamma RI, II and III on neutrophils and monocytes in 20 patients with KD, 10 with a bacterial infection (BI), 10 with a viral infection (VI), and 10 healthy controls (HC) using flow cytometric analysis. The KD patients had a significantly higher level of Fc gamma RI expression on neutrophils, but not on monocytes, than the BI, VI and HC patients. Fc gamma RII expression on neutrophils was significantly higher in KD, BI and VI than HC, but there was no significant difference in Fc gamma RII expression among KD, BI and VI. Fc gamma RIII expression on neutrophils in KD was significantly lower than in VI and HC, but was higher on monocytes. A kinetic analysis of Fc gamma R expression in KD demonstrated the expression of Fc gamma RI and II on neutrophils to decline, but no remarkable change was observed in the monocytes, from the subacute phase through the convalescent phase. In addition, Fc gamma RIII expression on neutrophils increased, while Fc gamma RIII expression on monocytes decreased during the time course of KD. Fc gamma R expression in the acute phase of KD is thus characterized by markedly increased expression of Fc gamma RI on neutrophils, followed by a subsequent decrease, and decreased expression of Fc gamma RIII on neutrophils and increased expression of Fc gamma RIII on monocytes followed by a reverse kinetics during the clinical course. These findings are thus considered to reflect the functional up-regulation of neutrophils and monocytes in KD. (+info)Staphylococcus aureus isolates from patients with Kawasaki disease express high levels of protein A. (6/526)
Kawasaki disease (KD) is an acute vasculitis of young children that can be complicated by coronary artery abnormalities. Recent findings suggest that a superantigen(s) may play an important role in stimulating the immune activation associated with the disease, although the origin of this superantigen(s) is unclear. Staphylococcus aureus, isolated from the rectum or pharynx of patients with KD, secretes toxic shock syndrome toxin 1 (TSST-1). The KD isolates express low levels of other exoproteins compared to isolates from patients with toxic shock syndrome (TSS). Thus, it was previously suggested that the KD isolates may be defective in the global regulatory locus agr (for accessory gene regulator), which positively regulates these factors (D. Y. M. Leung et al., Lancet 342:1385-1388, 1993). Here we describe another characteristic of KD isolates. When considered collectively, the KD isolates were found to express higher levels of staphylococcal protein A than the TSS isolates, another characteristic of an agr-defective phenotype. This correlated with a higher level of spa mRNA in these isolates. In contrast, the KD and TSS isolates expressed comparable levels of TSST-1, consistent with previous findings (D. Y. M. Leung et al., Lancet 342:1385-1388, 1993). Analysis of RNAIII transcript levels and nucleotide sequence analysis of the RNAIII-coding region suggested that the KD isolates are not defective in RNAIII, the effector molecule of the agr regulatory system. However, induction of RNAIII transcription in the KD isolates did not result in a dramatic decrease in the amount of spa mRNA, as has been reported for other strains (F. Vandenesch, J. Kornblum, and R. P. Novick, J. Bacteriol. 173:6313-6320, 1991). (+info)Changes in endothelium-derived vascular regulatory factors during dobutamine-stress-induced silent myocardial ischemia in patients with Kawasaki disease. (7/526)
The changes in endothelium-derived vascular regulatory factors during dobutamine (DOB)-induced myocardial ischemia (MI) were investigated in 21 patients with Kawasaki disease aged from 11 months to 18 years. They were classified into an ischemia group (8 patients) and a non-ischemia group (13 patients) based on the results of 99mTc myocardial scintigraphy and DOB stress 99mTc myocardial scintigraphy. In the ischemia group, MI was relatively mild, because there were ischemic changes on the electrocardiogram and no significant symptoms during DOB stress. Catheters were positioned near the orifice of the coronary artery (Ao) and at the coronary sinus (CS). Hemodynamics and the blood concentrations of lactic acid and endothelin-1, as well as NO3-, 6-keto-prostaglandin F1alpha, and thromboxane B2, (which are inactive metabolites of nitric oxide, prostaglandin I2 and thromboxane A2, respectively), were measured at rest and after DOB stress (maximum dose: 30 microg x kg(-1) x min(-1)). The CS/Ao ratio was determined for all parameters. The rate-pressure product, an index of work load, and the cardiac index were significantly increased by DOB stress in both groups. Coronary angiography showed no vasospasm of the epicardial coronary arteries before or after DOB stress in either group. The plasma concentrations of endothelin-1 and 6-keto-prostaglandin F1alpha were significantly increased after DOB stress in the ischemia group, but the serum concentration of NO did not increase. The lack of an increase in NO production during DOB stress may have contributed to the worsening of MI in patients with Kawasaki disease. (+info)Kawasaki syndrome-like illness associated with infection caused by enterotoxin B-secreting Staphylococcus aureus. (8/526)
Two children had symptoms and clinical signs that were characteristic of the diagnostic criteria for Kawasaki syndrome, temporally associated with Staphylococcus aureus bacteremia. One child initially had focal osteomyelitis that was evident clinically and radiographically, and radiographic evidence of multifocal osteomyelitis was noted at follow-up. The blood-borne S. aureus isolates from these two patients secreted staphylococcal enterotoxin B and were negative for toxic shock syndrome toxin. Staphylococcal and streptococcal superantigens may play a role in the pathogenesis of some cases of Kawasaki syndrome or Kawasaki syndrome-like illness. (+info)Mucocutaneous Lymph Node Syndrome is also known as Kawasaki Disease. It is a type of vasculitis that primarily affects young children, usually those under the age of 5. The disease is named after Dr. Tomisaku Kawasaki, who first described it in Japan in 1967.
The condition is characterized by inflammation of the mucous membranes (mucosa), skin (cutaneous), and lymph nodes. The symptoms typically include fever, rash, red eyes, swollen lips and tongue, strawberry tongue, and swollen lymph nodes in the neck. In addition, children with Kawasaki disease may also experience joint pain, diarrhea, vomiting, and abdominal pain.
In severe cases, Kawasaki disease can lead to complications such as coronary artery aneurysms, which can increase the risk of heart attacks and other cardiovascular problems. The exact cause of Kawasaki disease is unknown, but it is thought to be triggered by an infection or other environmental factor in genetically susceptible children. Treatment typically involves administering high doses of intravenous immunoglobulin (IVIG) and aspirin to reduce inflammation and prevent complications.
Lymphatic diseases refer to a group of conditions that affect the lymphatic system, which is an important part of the immune and circulatory systems. The lymphatic system consists of a network of vessels, organs, and tissues that help to transport lymph fluid throughout the body, fight infection, and remove waste products.
Lymphatic diseases can be caused by various factors, including genetics, infections, cancer, and autoimmune disorders. Some common types of lymphatic diseases include:
1. Lymphedema: A condition that causes swelling in the arms or legs due to a blockage or damage in the lymphatic vessels.
2. Lymphoma: A type of cancer that affects the lymphatic system, including Hodgkin's and non-Hodgkin's lymphoma.
3. Infections: Certain bacterial and viral infections can affect the lymphatic system, such as tuberculosis, cat-scratch disease, and HIV/AIDS.
4. Autoimmune disorders: Conditions such as rheumatoid arthritis, lupus, and scleroderma can cause inflammation and damage to the lymphatic system.
5. Congenital abnormalities: Some people are born with abnormalities in their lymphatic system, such as malformations or missing lymph nodes.
Symptoms of lymphatic diseases may vary depending on the specific condition and its severity. Treatment options may include medication, physical therapy, surgery, or radiation therapy. It is important to seek medical attention if you experience symptoms of a lymphatic disease, as early diagnosis and treatment can improve outcomes.
Lymph nodes are small, bean-shaped organs that are part of the immune system. They are found throughout the body, especially in the neck, armpits, groin, and abdomen. Lymph nodes filter lymph fluid, which carries waste and unwanted substances such as bacteria, viruses, and cancer cells. They contain white blood cells called lymphocytes that help fight infections and diseases by attacking and destroying the harmful substances found in the lymph fluid. When an infection or disease is present, lymph nodes may swell due to the increased number of immune cells and fluid accumulation as they work to fight off the invaders.
Chronic mucocutaneous candidiasis (CMC) is a group of rare disorders characterized by persistent or recurrent Candida infections of the skin, nails, and mucous membranes. The infection can affect various sites such as the mouth, esophagus, respiratory tract, gastrointestinal tract, and genitourinary tract.
CMC is typically caused by an impaired immune response to Candida albicans, a type of fungus that commonly exists on the skin and mucous membranes. In CMC, the immune system fails to control the growth of Candida, leading to chronic or recurrent infections.
The symptoms of CMC can vary depending on the site of infection. Common manifestations include:
* Chronic or recurrent thrush (oral candidiasis)
* Esophagitis (inflammation of the esophagus)
* Chronic nail infections (onychomycosis)
* Skin lesions, such as redness, swelling, and cracks
* Genital infections, including vaginitis and balanitis (inflammation of the head of the penis)
CMC can be associated with other immune disorders, such as endocrine dysfunction, autoimmune diseases, and primary immunodeficiencies. The diagnosis of CMC is based on clinical manifestations, laboratory tests, and imaging studies. Treatment typically involves antifungal medications, such as topical or systemic azoles, echinocandins, or polyenes. In some cases, immunomodulatory therapy may be necessary to manage the underlying immune dysfunction.
Mucocutaneous Leishmaniasis (MCL) is a chronic, granulomatous disease caused by an infection with Leishmania species, primarily L. braziliensis and L. guyanensis. It affects both the mucous membranes (such as those of the nose, mouth, and throat) and the skin.
The initial infection often occurs through the bite of an infected female sandfly, which transmits the parasitic protozoa into the host's skin. After a variable incubation period, the disease can manifest in different clinical forms, including localized cutaneous leishmaniasis (CL), disseminated cutaneous leishmaniasis, and mucocutaneous leishmaniasis.
MCL is characterized by progressive destruction of the mucous membranes, leading to deformities and functional impairments. The infection typically starts as a cutaneous lesion at the site of the sandfly bite, which heals spontaneously within several months. However, in some cases, the parasites disseminate to the mucous membranes, causing severe inflammation, ulceration, and tissue necrosis.
Symptoms of MCL include:
1. Destruction of nasal septum, leading to a saddle-nose deformity
2. Perforation of the palate or septum
3. Hoarseness or loss of voice due to laryngeal involvement
4. Difficulty swallowing and speaking
5. Chronic rhinitis, sinusitis, or otitis media
6. Severe disfigurement and functional impairments in advanced cases
Diagnosis is usually made by identifying the parasites in tissue samples (such as biopsies) using microscopy, culture, or PCR-based methods. Treatment typically involves systemic antiparasitic drugs, such as pentavalent antimonials, amphotericin B, miltefosine, or combination therapies, along with surgical interventions to reconstruct damaged tissues in advanced cases.
A coronary aneurysm is a localized dilation or bulging of a portion of the wall of a coronary artery, which supplies blood to the muscle tissue of the heart. It's similar to a bubble or balloon-like structure that forms within the artery wall due to weakness in the arterial wall, leading to abnormal enlargement or widening.
Coronary aneurysms can vary in size and may be classified as true or false aneurysms based on their structure. True aneurysms involve all three layers of the artery wall, while false aneurysms (also known as pseudoaneurysms) only have one or two layers involved, with the remaining layer disrupted.
These aneurysms can lead to complications such as blood clots forming inside the aneurysm sac, which can then dislodge and cause blockages in smaller coronary arteries (embolism). Additionally, coronary aneurysms may rupture, leading to severe internal bleeding and potentially life-threatening situations.
Coronary aneurysms are often asymptomatic but can present with symptoms such as chest pain, shortness of breath, or palpitations, especially if the aneurysm causes a significant narrowing (stenosis) in the affected artery. They can be diagnosed through imaging techniques like coronary angiography, computed tomography (CT), or magnetic resonance imaging (MRI). Treatment options include medications to manage symptoms and prevent complications, as well as surgical interventions such as stenting or bypass grafting to repair or reroute the affected artery.
Intravenous Immunoglobulins (IVIG) are a preparation of antibodies, specifically immunoglobulins, that are derived from the plasma of healthy donors. They are administered intravenously to provide passive immunity and help boost the immune system's response in individuals with weakened or compromised immune systems. IVIG can be used for various medical conditions such as primary immunodeficiency disorders, secondary immunodeficiencies, autoimmune diseases, and some infectious diseases. The administration of IVIG can help prevent infections, reduce the severity and frequency of infections, and manage the symptoms of certain autoimmune disorders. It is important to note that while IVIG provides temporary immunity, it does not replace a person's own immune system.
An encyclopedia is a comprehensive reference work containing articles on various topics, usually arranged in alphabetical order. In the context of medicine, a medical encyclopedia is a collection of articles that provide information about a wide range of medical topics, including diseases and conditions, treatments, tests, procedures, and anatomy and physiology. Medical encyclopedias may be published in print or electronic formats and are often used as a starting point for researching medical topics. They can provide reliable and accurate information on medical subjects, making them useful resources for healthcare professionals, students, and patients alike. Some well-known examples of medical encyclopedias include the Merck Manual and the Stedman's Medical Dictionary.
Coronary vessels refer to the network of blood vessels that supply oxygenated blood and nutrients to the heart muscle, also known as the myocardium. The two main coronary arteries are the left main coronary artery and the right coronary artery.
The left main coronary artery branches off into the left anterior descending artery (LAD) and the left circumflex artery (LCx). The LAD supplies blood to the front of the heart, while the LCx supplies blood to the side and back of the heart.
The right coronary artery supplies blood to the right lower part of the heart, including the right atrium and ventricle, as well as the back of the heart.
Coronary vessel disease (CVD) occurs when these vessels become narrowed or blocked due to the buildup of plaque, leading to reduced blood flow to the heart muscle. This can result in chest pain, shortness of breath, or a heart attack.
An aneurysm is a localized, balloon-like bulge in the wall of a blood vessel. It occurs when the pressure inside the vessel causes a weakened area to swell and become enlarged. Aneurysms can develop in any blood vessel, but they are most common in arteries at the base of the brain (cerebral aneurysm) and the main artery carrying blood from the heart to the rest of the body (aortic aneurysm).
Aneurysms can be classified as saccular or fusiform, depending on their shape. A saccular aneurysm is a round or oval bulge that projects from the side of a blood vessel, while a fusiform aneurysm is a dilated segment of a blood vessel that is uniform in width and involves all three layers of the arterial wall.
The size and location of an aneurysm can affect its risk of rupture. Generally, larger aneurysms are more likely to rupture than smaller ones. Aneurysms located in areas with high blood pressure or where the vessel branches are also at higher risk of rupture.
Ruptured aneurysms can cause life-threatening bleeding and require immediate medical attention. Symptoms of a ruptured aneurysm may include sudden severe headache, neck stiffness, nausea, vomiting, blurred vision, or loss of consciousness. Unruptured aneurysms may not cause any symptoms and are often discovered during routine imaging tests for other conditions.
Treatment options for aneurysms depend on their size, location, and risk of rupture. Small, unruptured aneurysms may be monitored with regular imaging tests to check for growth or changes. Larger or symptomatic aneurysms may require surgical intervention, such as clipping or coiling, to prevent rupture and reduce the risk of complications.
An intracranial aneurysm is a localized, blood-filled dilation or bulging in the wall of a cerebral artery within the skull (intracranial). These aneurysms typically occur at weak points in the arterial walls, often at branching points where the vessel divides into smaller branches. Over time, the repeated pressure from blood flow can cause the vessel wall to weaken and balloon out, forming a sac-like structure. Intracranial aneurysms can vary in size, ranging from a few millimeters to several centimeters in diameter.
There are three main types of intracranial aneurysms:
1. Saccular (berry) aneurysm: This is the most common type, characterized by a round or oval shape with a narrow neck and a bulging sac. They usually develop at branching points in the arteries due to congenital weaknesses in the vessel wall.
2. Fusiform aneurysm: These aneurysms have a dilated segment along the length of the artery, forming a cigar-shaped or spindle-like structure. They are often caused by atherosclerosis and can affect any part of the cerebral arteries.
3. Dissecting aneurysm: This type occurs when there is a tear in the inner lining (intima) of the artery, allowing blood to flow between the layers of the vessel wall. It can lead to narrowing or complete blockage of the affected artery and may cause subarachnoid hemorrhage if it ruptures.
Intracranial aneurysms can be asymptomatic and discovered incidentally during imaging studies for other conditions. However, when they grow larger or rupture, they can lead to severe complications such as subarachnoid hemorrhage, stroke, or even death. Treatment options include surgical clipping, endovascular coiling, or flow diversion techniques to prevent further growth and potential rupture of the aneurysm.
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