Kawasaki disease is an acute childhood vasculitis that can cause coronary artery abnormalities. It presents with fever and changes in lips, oral cavity, hands/feet, rash and lymphadenopathy. Echocardiogram monitors for coronary complications like aneurysms. Treatment is high dose IVIG and aspirin to reduce inflammation and risk of coronary abnormalities. Some children are resistant to initial treatment and require additional immunosuppressants. Long term low dose aspirin helps prevent complications in those with coronary abnormalities.
KAWASAKI DISEASE
History of Kawasaki disease
Epidemiology and etiology
Presentation and diagnosis
Treatment
Chronic cardiovascular manifestations
Follow up of patients
Questions in the chronic management
This document provides an overview of Kawasaki disease, including its history, definition, epidemiology, pathogenesis, clinical features, diagnosis, differential diagnosis, complications, and treatment. Kawasaki disease is an acute febrile illness that predominantly affects children under 5 years old and can lead to coronary artery aneurysms if left untreated. It is diagnosed based on the presence of fever for at least 5 days along with four of five principal clinical features. Intravenous immunoglobulin and aspirin are the primary treatments used to reduce the risk of coronary complications.
Kawasaki disease is an acute febrile illness that primarily affects children under 5 years old. It is characterized by vasculitis and inflammation of blood vessels, especially the coronary arteries. While the cause is unknown, it likely has an infectious and genetic component. If left untreated, it can lead to coronary artery aneurysms in about 25% of cases. Treatment involves intravenous immunoglobulin and aspirin to reduce inflammation and risk of aneurysms developing. Prognosis is generally good if treated promptly, but giant coronary aneurysms carry a risk of thrombosis, stenosis, and myocardial infarction if not closely monitored long-term.
This document presents a case study of a 3-year-old female patient admitted with fever, skin rash, mouth ulcers, and red eyes. After initial treatment for a suspected viral infection, her symptoms worsened with swelling of the hands and feet. She was diagnosed with possible Kawasaki disease and treated with intravenous immunoglobulin and aspirin. Her fever subsided after treatment but returned, requiring a second dose of IVIG. She was discharged after 10 days against medical advice with aspirin treatment and follow-up appointments. Kawasaki disease is an acute childhood vasculitis of unknown cause seen more in Asian children. It involves fever, rash, mouth changes, conjunctivitis, swelling of hands
This document discusses the diagnosis, treatment, and long-term management of Kawasaki disease. It begins with an overview of the epidemiology of the disease, including that it predominantly affects children under 5 years old and has the highest incidence in Japan, Korea, and Taiwan. It then covers the pathology and causes of the disease, which involves a systemic vasculitis of medium-sized arteries. Three linked pathological processes are described: necrotizing arteritis in the initial weeks, followed by subacute/chronic vasculitis and luminal myofibroblastic proliferation that can persist for months/years and cause coronary artery aneurysms or stenosis. The document outlines the diagnostic criteria for Kawasaki disease as a high
This document provides an overview of Kawasaki Disease including its history, definition, epidemiology, etiology, diagnostic criteria, cardiac and non-cardiac findings, treatment, complications, and references. Kawasaki Disease is an autoimmune mediated vasculitis that commonly affects children under 5 years old and can lead to coronary artery abnormalities if left untreated. Treatment involves intravenous immunoglobulin and aspirin to reduce complications. Long term risks include coronary artery aneurysms and stenosis.
Kawasaki disease is a childhood vasculitis that causes inflammation in blood vessels. It is characterized by fever and rash. If not treated, it can lead to inflammation of arteries of the heart. The cause is unknown. Treatment involves intravenous immunoglobulin which reduces risk of heart complications if given early. Long term risks include heart disease if large arteries of the heart are affected during the initial infection. Prognosis is generally good if treated promptly.
Kawasaki disease is an inflammation of blood vessels that mainly affects children under 5 years old. It is the leading cause of acquired heart disease in children. While the cause is unknown, it is suspected to be a virus. If not diagnosed within 10 days of symptoms appearing, there is an increased risk of heart problems developing. The disease involves fever, rash, swelling of hands and feet, and inflammation of mucous membranes. Without treatment, 1% of children with Kawasaki disease can die from it and aneurysms in the coronary arteries can develop which may lead to heart attacks later in life.
KAWASAKI DISEASE
History of Kawasaki disease
Epidemiology and etiology
Presentation and diagnosis
Treatment
Chronic cardiovascular manifestations
Follow up of patients
Questions in the chronic management
This document provides an overview of Kawasaki disease, including its history, definition, epidemiology, pathogenesis, clinical features, diagnosis, differential diagnosis, complications, and treatment. Kawasaki disease is an acute febrile illness that predominantly affects children under 5 years old and can lead to coronary artery aneurysms if left untreated. It is diagnosed based on the presence of fever for at least 5 days along with four of five principal clinical features. Intravenous immunoglobulin and aspirin are the primary treatments used to reduce the risk of coronary complications.
Kawasaki disease is an acute febrile illness that primarily affects children under 5 years old. It is characterized by vasculitis and inflammation of blood vessels, especially the coronary arteries. While the cause is unknown, it likely has an infectious and genetic component. If left untreated, it can lead to coronary artery aneurysms in about 25% of cases. Treatment involves intravenous immunoglobulin and aspirin to reduce inflammation and risk of aneurysms developing. Prognosis is generally good if treated promptly, but giant coronary aneurysms carry a risk of thrombosis, stenosis, and myocardial infarction if not closely monitored long-term.
This document presents a case study of a 3-year-old female patient admitted with fever, skin rash, mouth ulcers, and red eyes. After initial treatment for a suspected viral infection, her symptoms worsened with swelling of the hands and feet. She was diagnosed with possible Kawasaki disease and treated with intravenous immunoglobulin and aspirin. Her fever subsided after treatment but returned, requiring a second dose of IVIG. She was discharged after 10 days against medical advice with aspirin treatment and follow-up appointments. Kawasaki disease is an acute childhood vasculitis of unknown cause seen more in Asian children. It involves fever, rash, mouth changes, conjunctivitis, swelling of hands
This document discusses the diagnosis, treatment, and long-term management of Kawasaki disease. It begins with an overview of the epidemiology of the disease, including that it predominantly affects children under 5 years old and has the highest incidence in Japan, Korea, and Taiwan. It then covers the pathology and causes of the disease, which involves a systemic vasculitis of medium-sized arteries. Three linked pathological processes are described: necrotizing arteritis in the initial weeks, followed by subacute/chronic vasculitis and luminal myofibroblastic proliferation that can persist for months/years and cause coronary artery aneurysms or stenosis. The document outlines the diagnostic criteria for Kawasaki disease as a high
This document provides an overview of Kawasaki Disease including its history, definition, epidemiology, etiology, diagnostic criteria, cardiac and non-cardiac findings, treatment, complications, and references. Kawasaki Disease is an autoimmune mediated vasculitis that commonly affects children under 5 years old and can lead to coronary artery abnormalities if left untreated. Treatment involves intravenous immunoglobulin and aspirin to reduce complications. Long term risks include coronary artery aneurysms and stenosis.
Kawasaki disease is a childhood vasculitis that causes inflammation in blood vessels. It is characterized by fever and rash. If not treated, it can lead to inflammation of arteries of the heart. The cause is unknown. Treatment involves intravenous immunoglobulin which reduces risk of heart complications if given early. Long term risks include heart disease if large arteries of the heart are affected during the initial infection. Prognosis is generally good if treated promptly.
Kawasaki disease is an inflammation of blood vessels that mainly affects children under 5 years old. It is the leading cause of acquired heart disease in children. While the cause is unknown, it is suspected to be a virus. If not diagnosed within 10 days of symptoms appearing, there is an increased risk of heart problems developing. The disease involves fever, rash, swelling of hands and feet, and inflammation of mucous membranes. Without treatment, 1% of children with Kawasaki disease can die from it and aneurysms in the coronary arteries can develop which may lead to heart attacks later in life.
Kawasaki disease is an acute self-limited vasculitis that primarily affects children under 5 years old. It is characterized by prolonged fever and changes in the mouth, hands and feet, skin rash, and blood vessel inflammation that can lead to aneurysm formation in untreated cases. While the cause is unknown, it is believed to be infectious and may be triggered by a combination of genetic and environmental factors. Prompt treatment with intravenous immunoglobulin can reduce the risk of coronary artery aneurysms and subsequent heart disease.
This document presents a case study of a 2-year-old female infant diagnosed with Kawasaki disease based on symptoms of fever, swollen lips, and ankle swelling. The patient was treated with intravenous immunoglobulin and aspirin, showed improvement, and was discharged with a final diagnosis of incomplete Kawasaki disease. The document then provides a brief overview of Kawasaki disease, including epidemiology, pathophysiology, diagnostic criteria, treatment, and potential complications like coronary artery aneurysms and long-term cardiac issues.
Here are the answers:
A. True
B. False (IVIG and low dose aspirin used)
C. False
D. True
E. False (most common <5 years)
T/F regarding Kawasaki disease
- True
- True
- True
- True
- True
Kawasaki disease is an autoimmune disease that causes inflammation in blood vessels throughout the body. It was first described in 1967 by Dr. Kawasaki in Japan. It most commonly affects children under 5 years old. Without treatment, it can lead to fatal coronary artery aneurysms in some children. The cause is unknown but likely involves genetic and environmental factors such as a viral or bacterial infection. Diagnosis is based on symptoms that include prolonged fever and changes in lips, mouth, hands and feet. Echocardiograms are used to check for heart complications which include aneurysms and heart valve issues.
Kawasaki disease is an acute vasculitis that predominantly affects coronary arteries in children. It is characterized by fever, rash, conjunctival injection, cervical lymphadenopathy, and changes to the mouth and lips. The cause is unknown but may involve a novel RNA virus. Treatment involves intravenous immunoglobulin and aspirin to reduce inflammation and prevent coronary artery abnormalities. Refractory cases may require additional immunomodulating therapies.
Kawasaki Disease is a self-limited vasculitis that predominantly affects children under 5 years old and can lead to coronary artery aneurysms if left untreated; it is characterized by fever for at least 5 days along with changes in extremities, rash, conjunctival injection, and cervical lymphadenopathy. Intravenous immunoglobulin and aspirin are the primary treatment to reduce the risk of coronary artery aneurysms developing.
1) Kawasaki disease is an acute self-limited vasculitis that commonly affects children under 5 years old, especially of Japanese ancestry. It is characterized by fever, rash, conjunctivitis, changes in the mouth and lips, swelling of hands and feet, and cervical lymphadenopathy.
2) If left untreated, Kawasaki disease can lead to coronary artery aneurysms in 15-25% of children, which increases the risk of heart attacks and sudden cardiac death later in life.
3) While the exact cause is unknown, it is believed to be an infectious agent that triggers an immunological response in genetically susceptible children. Treatment involves intravenous immunoglobulin and aspirin to reduce inflammation
Kawasaki disease is a multisystem vasculitis that predominantly affects children under 5 years old. It is characterized by inflammation of blood vessels including the coronary arteries. While the cause is unknown, it is believed to be infectious. Symptoms include prolonged fever, rash, conjunctivitis, swelling of hands and feet, and lymphadenopathy. Treatment involves high dose aspirin and intravenous immunoglobulin to prevent coronary artery aneurysms which can lead to complications like heart attacks if not treated early. With treatment, most children fully recover but lifelong cardiology monitoring is important for those with cardiac involvement.
The document describes a case study of a 4-year-old child presenting with intermittent fever, cough, weakness, and decreased appetite for 20 days. Examination reveals enlarged lymph nodes and crepitations. Differential diagnoses include tuberculosis, pneumonia, and other conditions. The most likely diagnosis is tuberculosis given the child's exposure to a grandfather with chronic cough, incomplete vaccinations, and findings on examination. Proper management includes further investigations and standard antituberculosis treatment regimens. The document then discusses tuberculosis in children more broadly, including causative agents, transmission, clinical features, investigations, treatment, drug-resistant tuberculosis, and prevention through BCG vaccination.
This document discusses a case of Kawasaki disease in a 2-year-old boy presenting with fever and limp. It then provides details on Kawasaki disease including that it is the most common cause of acquired heart disease in children, characterized by inflammation of blood vessels. Signs and symptoms, diagnostic criteria, differential diagnosis, cardiovascular manifestations, and management are described. The importance of early diagnosis and treatment to prevent cardiac complications is emphasized.
This document discusses fever without a clear source or focus of infection. It defines fever and describes how body temperature is regulated. Fever occurs when heat production exceeds heat loss. Common causes include infections, inflammation, and cancer. The pattern of fever can provide clues to the underlying cause. Management depends on the age of the child. For young infants, a full evaluation including lab tests and antibiotics is often needed due to the risk of serious bacterial infection. Older children have a lower risk but may still require testing and observation or empiric antibiotics depending on factors like vaccination history and appearance.
This document summarizes Kawasaki disease, an acute febrile illness that causes vasculitis of medium-sized arteries, especially coronary arteries. It can lead to coronary artery aneurysms and thrombosis. The disease is diagnosed based on clinical criteria in the absence of a confirmatory test. Treatment with intravenous immunoglobulin can reduce the risk of coronary artery lesions from 25% to 3-5%, but infants and incomplete presentations remain at higher risk.
The patient, a 23-year-old male, presented with abdominal pain and was found to have palpable purpura on his lower extremities. He was initially diagnosed with acute appendicitis but developed a skin rash. Biopsy of the skin rash showed leukocytoclastic vasculitis. He was ultimately diagnosed with Henoch-Schonlein purpura (HSP), characterized by palpable purpura, arthritis/arthralgia, gastrointestinal involvement, and renal involvement. HSP is an immune complex-mediated vasculitis that commonly affects children and is generally self-limiting, though steroids may help with symptoms. Prognosis depends on factors like age of onset, severity
This document provides guidance on evaluating a child presenting with fever and rash. It describes the key characteristics of fever and rash, important aspects of history and physical exam, and the differential diagnosis for common infectious and inflammatory causes of fever and rash in children. These include viral illnesses like measles, chickenpox, rubella, scarlet fever, dengue fever, and typhoid fever, as well as bacterial infections like Kawasaki disease, systemic lupus erythematosus, and infectious mononucleosis. Diagnosis and treatment options are outlined for each condition. A thorough history, physical exam focusing on rash characteristics, and diagnostic testing can help identify the underlying cause.
This document provides information about Familial Mediterranean Fever (FMF), including that it is a genetic autoinflammatory disease characterized by recurrent attacks of fever and pain. If untreated, it can lead to amyloidosis and kidney failure. The recommended treatment is lifelong use of colchicine to prevent attacks and amyloidosis.
A 3-year-old girl presented with 5 days of fever, abdominal pain, vomiting, and rash. Her symptoms included conjunctivitis, cracked lips, cervical lymphadenopathy, and skin changes. Laboratory findings showed elevated inflammatory markers, hypoalbuminemia, and elevated liver enzymes. Echocardiogram found decreased left ventricular function and pericardial effusion. She was diagnosed with incomplete Kawasaki disease and treated with IVIG and aspirin, with improvement of symptoms and cardiac function. Consideration of Kawasaki disease is important for prolonged fever, especially in infants under 6 months old.
This document discusses rheumatic fever in children. It provides an overview of the incidence and prevalence of rheumatic fever worldwide and in Nepal. It describes the pathogenesis of rheumatic fever as an autoimmune response to Group A streptococcal infection. The document outlines the criteria for diagnosing rheumatic fever, including Jones criteria and WHO criteria. It also discusses treatment approaches such as aspirin to suppress inflammation.
The document provides descriptions of 10 common pediatric rash cases. Each case includes the causative agent, characteristics of the rash, treatment, and any complications. The rashes described are measles, roseola, impetigo, tinea corporis, candida dermatitis, scabies, scarlet fever, atopic dermatitis, varicella, and erythema infectiosum. The document serves to familiarize medical professionals with the presentations of common rashes in children so they can differentiate between diagnoses and provide appropriate treatment.
Kawasaki Disease (KD) is an acute inflammatory syndrome that predominantly affects children. It is characterized by fever and changes in the mouth, hands, and feet. Important complications include coronary artery dilation and aneurysm formation if left untreated. While the cause is unknown, it is suspected to be infectious in nature and to involve an immunological component. Treatment aims to reduce the risk of heart complications and involves high doses of intravenous immunoglobulin and aspirin.
This document provides an overview of Kawasaki disease, including:
- It is a vasculitis that predominantly affects children under 5 years old and can lead to coronary artery aneurysms if left untreated.
- Diagnosis is based on fever for at least 5 days along with 4 out of 5 criteria involving mucocutaneous changes and lymph node swelling.
- Treatment involves intravenous immunoglobulin and high-dose aspirin which significantly reduces the risk of coronary artery abnormalities compared to aspirin alone.
- Ongoing monitoring is needed due to the potential for coronary artery aneurysms and increased risk of heart issues like myocardial infarction in the future.
Kawasaki disease is an acute self-limited vasculitis that primarily affects children under 5 years old. It is characterized by prolonged fever and changes in the mouth, hands and feet, skin rash, and blood vessel inflammation that can lead to aneurysm formation in untreated cases. While the cause is unknown, it is believed to be infectious and may be triggered by a combination of genetic and environmental factors. Prompt treatment with intravenous immunoglobulin can reduce the risk of coronary artery aneurysms and subsequent heart disease.
This document presents a case study of a 2-year-old female infant diagnosed with Kawasaki disease based on symptoms of fever, swollen lips, and ankle swelling. The patient was treated with intravenous immunoglobulin and aspirin, showed improvement, and was discharged with a final diagnosis of incomplete Kawasaki disease. The document then provides a brief overview of Kawasaki disease, including epidemiology, pathophysiology, diagnostic criteria, treatment, and potential complications like coronary artery aneurysms and long-term cardiac issues.
Here are the answers:
A. True
B. False (IVIG and low dose aspirin used)
C. False
D. True
E. False (most common <5 years)
T/F regarding Kawasaki disease
- True
- True
- True
- True
- True
Kawasaki disease is an autoimmune disease that causes inflammation in blood vessels throughout the body. It was first described in 1967 by Dr. Kawasaki in Japan. It most commonly affects children under 5 years old. Without treatment, it can lead to fatal coronary artery aneurysms in some children. The cause is unknown but likely involves genetic and environmental factors such as a viral or bacterial infection. Diagnosis is based on symptoms that include prolonged fever and changes in lips, mouth, hands and feet. Echocardiograms are used to check for heart complications which include aneurysms and heart valve issues.
Kawasaki disease is an acute vasculitis that predominantly affects coronary arteries in children. It is characterized by fever, rash, conjunctival injection, cervical lymphadenopathy, and changes to the mouth and lips. The cause is unknown but may involve a novel RNA virus. Treatment involves intravenous immunoglobulin and aspirin to reduce inflammation and prevent coronary artery abnormalities. Refractory cases may require additional immunomodulating therapies.
Kawasaki Disease is a self-limited vasculitis that predominantly affects children under 5 years old and can lead to coronary artery aneurysms if left untreated; it is characterized by fever for at least 5 days along with changes in extremities, rash, conjunctival injection, and cervical lymphadenopathy. Intravenous immunoglobulin and aspirin are the primary treatment to reduce the risk of coronary artery aneurysms developing.
1) Kawasaki disease is an acute self-limited vasculitis that commonly affects children under 5 years old, especially of Japanese ancestry. It is characterized by fever, rash, conjunctivitis, changes in the mouth and lips, swelling of hands and feet, and cervical lymphadenopathy.
2) If left untreated, Kawasaki disease can lead to coronary artery aneurysms in 15-25% of children, which increases the risk of heart attacks and sudden cardiac death later in life.
3) While the exact cause is unknown, it is believed to be an infectious agent that triggers an immunological response in genetically susceptible children. Treatment involves intravenous immunoglobulin and aspirin to reduce inflammation
Kawasaki disease is a multisystem vasculitis that predominantly affects children under 5 years old. It is characterized by inflammation of blood vessels including the coronary arteries. While the cause is unknown, it is believed to be infectious. Symptoms include prolonged fever, rash, conjunctivitis, swelling of hands and feet, and lymphadenopathy. Treatment involves high dose aspirin and intravenous immunoglobulin to prevent coronary artery aneurysms which can lead to complications like heart attacks if not treated early. With treatment, most children fully recover but lifelong cardiology monitoring is important for those with cardiac involvement.
The document describes a case study of a 4-year-old child presenting with intermittent fever, cough, weakness, and decreased appetite for 20 days. Examination reveals enlarged lymph nodes and crepitations. Differential diagnoses include tuberculosis, pneumonia, and other conditions. The most likely diagnosis is tuberculosis given the child's exposure to a grandfather with chronic cough, incomplete vaccinations, and findings on examination. Proper management includes further investigations and standard antituberculosis treatment regimens. The document then discusses tuberculosis in children more broadly, including causative agents, transmission, clinical features, investigations, treatment, drug-resistant tuberculosis, and prevention through BCG vaccination.
This document discusses a case of Kawasaki disease in a 2-year-old boy presenting with fever and limp. It then provides details on Kawasaki disease including that it is the most common cause of acquired heart disease in children, characterized by inflammation of blood vessels. Signs and symptoms, diagnostic criteria, differential diagnosis, cardiovascular manifestations, and management are described. The importance of early diagnosis and treatment to prevent cardiac complications is emphasized.
This document discusses fever without a clear source or focus of infection. It defines fever and describes how body temperature is regulated. Fever occurs when heat production exceeds heat loss. Common causes include infections, inflammation, and cancer. The pattern of fever can provide clues to the underlying cause. Management depends on the age of the child. For young infants, a full evaluation including lab tests and antibiotics is often needed due to the risk of serious bacterial infection. Older children have a lower risk but may still require testing and observation or empiric antibiotics depending on factors like vaccination history and appearance.
This document summarizes Kawasaki disease, an acute febrile illness that causes vasculitis of medium-sized arteries, especially coronary arteries. It can lead to coronary artery aneurysms and thrombosis. The disease is diagnosed based on clinical criteria in the absence of a confirmatory test. Treatment with intravenous immunoglobulin can reduce the risk of coronary artery lesions from 25% to 3-5%, but infants and incomplete presentations remain at higher risk.
The patient, a 23-year-old male, presented with abdominal pain and was found to have palpable purpura on his lower extremities. He was initially diagnosed with acute appendicitis but developed a skin rash. Biopsy of the skin rash showed leukocytoclastic vasculitis. He was ultimately diagnosed with Henoch-Schonlein purpura (HSP), characterized by palpable purpura, arthritis/arthralgia, gastrointestinal involvement, and renal involvement. HSP is an immune complex-mediated vasculitis that commonly affects children and is generally self-limiting, though steroids may help with symptoms. Prognosis depends on factors like age of onset, severity
This document provides guidance on evaluating a child presenting with fever and rash. It describes the key characteristics of fever and rash, important aspects of history and physical exam, and the differential diagnosis for common infectious and inflammatory causes of fever and rash in children. These include viral illnesses like measles, chickenpox, rubella, scarlet fever, dengue fever, and typhoid fever, as well as bacterial infections like Kawasaki disease, systemic lupus erythematosus, and infectious mononucleosis. Diagnosis and treatment options are outlined for each condition. A thorough history, physical exam focusing on rash characteristics, and diagnostic testing can help identify the underlying cause.
This document provides information about Familial Mediterranean Fever (FMF), including that it is a genetic autoinflammatory disease characterized by recurrent attacks of fever and pain. If untreated, it can lead to amyloidosis and kidney failure. The recommended treatment is lifelong use of colchicine to prevent attacks and amyloidosis.
A 3-year-old girl presented with 5 days of fever, abdominal pain, vomiting, and rash. Her symptoms included conjunctivitis, cracked lips, cervical lymphadenopathy, and skin changes. Laboratory findings showed elevated inflammatory markers, hypoalbuminemia, and elevated liver enzymes. Echocardiogram found decreased left ventricular function and pericardial effusion. She was diagnosed with incomplete Kawasaki disease and treated with IVIG and aspirin, with improvement of symptoms and cardiac function. Consideration of Kawasaki disease is important for prolonged fever, especially in infants under 6 months old.
This document discusses rheumatic fever in children. It provides an overview of the incidence and prevalence of rheumatic fever worldwide and in Nepal. It describes the pathogenesis of rheumatic fever as an autoimmune response to Group A streptococcal infection. The document outlines the criteria for diagnosing rheumatic fever, including Jones criteria and WHO criteria. It also discusses treatment approaches such as aspirin to suppress inflammation.
The document provides descriptions of 10 common pediatric rash cases. Each case includes the causative agent, characteristics of the rash, treatment, and any complications. The rashes described are measles, roseola, impetigo, tinea corporis, candida dermatitis, scabies, scarlet fever, atopic dermatitis, varicella, and erythema infectiosum. The document serves to familiarize medical professionals with the presentations of common rashes in children so they can differentiate between diagnoses and provide appropriate treatment.
Kawasaki Disease (KD) is an acute inflammatory syndrome that predominantly affects children. It is characterized by fever and changes in the mouth, hands, and feet. Important complications include coronary artery dilation and aneurysm formation if left untreated. While the cause is unknown, it is suspected to be infectious in nature and to involve an immunological component. Treatment aims to reduce the risk of heart complications and involves high doses of intravenous immunoglobulin and aspirin.
This document provides an overview of Kawasaki disease, including:
- It is a vasculitis that predominantly affects children under 5 years old and can lead to coronary artery aneurysms if left untreated.
- Diagnosis is based on fever for at least 5 days along with 4 out of 5 criteria involving mucocutaneous changes and lymph node swelling.
- Treatment involves intravenous immunoglobulin and high-dose aspirin which significantly reduces the risk of coronary artery abnormalities compared to aspirin alone.
- Ongoing monitoring is needed due to the potential for coronary artery aneurysms and increased risk of heart issues like myocardial infarction in the future.
This document defines and describes different types of vasculitis. It begins by defining vasculitis as inflammation of blood vessel walls. The two main ways of classifying vasculitides are by the size of blood vessels involved and the presence or absence of ANCA. Small vessel vasculitis can be ANCA-positive (e.g. Wegener's granulomatosis, Churg-Strauss syndrome) or ANCA-negative (e.g. Henoch-Schönlein purpura). Medium vessel vasculitides include polyarteritis nodosa and Kawasaki's disease. Large vessel vasculitides include giant cell arteritis, Takayasaki's disease, and poly
Pulmonary arterial hypertension (PAH) in ccongenital heart diseasesMalleswara rao Dangeti
1. Pulmonary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure >25 mm Hg at rest or >30 mm Hg with exercise due to abnormalities in the pulmonary vasculature.
2. PAH can develop in patients with congenital heart disease (CHD) such as ventricular septal defects due to persistent high pulmonary blood flow leading to vascular changes over time.
3. In advanced PAH associated with CHD, known as Eisenmenger syndrome, pulmonary vascular resistance rises to high levels, equalizing pressures in the pulmonary and systemic circulations and resulting in a reversed or bidirectional shunt.
The document summarizes principles of electrocardiography and rheumatic fever. It discusses:
1. The electrocardiogram represents electrical activity of the heart during the cardiac cycle, starting from the SA node through the ventricles. Standard ECG analysis examines heart rate, rhythm, intervals and abnormalities.
2. Rheumatic fever is caused by untreated streptococcal infection and can lead to heart damage. It is diagnosed using Jones Criteria involving major criteria of carditis, arthritis, chorea and minor criteria including fever and joint pain. Treatment involves antibiotics and anti-inflammatories.
3. Mitral stenosis is most often caused by rheumatic heart disease involving thickened,
Acute rheumatic fever is an autoimmune disorder triggered by Group A streptococcal infection that causes inflammation in the heart, joints, brain, and skin. It most commonly affects children ages 5-15. Symptoms include fever, joint pain, and heart complications. Diagnosis involves evidence of a prior streptococcal infection and either two major symptoms or one major and two minor symptoms. Long-term antibiotic prophylaxis is needed to prevent recurrence and further heart damage. Without treatment, acute rheumatic fever can lead to rheumatic heart disease.
Anaesthetic management of a patient with mitral stenosis put for non-cardiac ...Ankur Khandelwal
Mitral stenosis is a narrowing of the mitral valve that causes obstruction of blood flow from the left atrium to the left ventricle. Rheumatic fever is the most common cause. Symptoms range from none in mild cases, to shortness of breath with exertion in moderate cases, to shortness of breath at rest in severe cases. Diagnosis is made through echocardiogram which can assess the severity based on metrics like mitral valve area and pressure gradients. Treatment depends on symptoms and severity, ranging from medications and lifestyle changes in mild cases, to balloon valvuloplasty or surgical commissurotomy in moderate to severe cases. Anesthetic management aims to avoid tachycardia and
Kawasaki disease is an acute febrile vasculitis that commonly affects children under 5 years old. It is characterized by fever, rash, conjunctivitis, oral changes and extremity changes. If left untreated it can lead to coronary artery aneurysms in 15-25% of cases. The cause is unknown but believed to be due to an infectious trigger in genetically susceptible children. Treatment involves intravenous immunoglobulin and aspirin to reduce the risk of coronary complications. Ongoing monitoring is needed due to the potential for aneurysms and subsequent cardiac issues.
Takayasu arteritis is a chronic inflammatory disease that causes stenosis, occlusion, dilation or aneurysm of the aorta and its branches. It most commonly affects adolescent girls and young women. Symptoms include limb claudication, decreased brachial pulse, hypertension, bruits, and vascular ischemia. Diagnosis is based on meeting criteria such as age of onset under 40, decreased pulse, blood pressure difference between arms, and angiographic evidence of vascular involvement. Treatment involves glucocorticoids which can control inflammation and symptoms, though relapses may occur. Additional immunosuppressants may be needed, and management of hypertension is important, especially during pregnancy which carries higher risks but fertility is not affected.
1) Rheumatic fever is an autoimmune disease that can occur as a delayed complication of untreated Group A streptococcal pharyngitis, with a latent period of 1-3 weeks.
2) It commonly affects children between 5-15 years of age and can involve the heart, joints, skin, and brain. The heart is involved in approximately 50-60% of cases (carditis).
3) Treatment involves bed rest, antibiotics to eradicate streptococci, anti-inflammatory drugs like aspirin for arthritis and carditis, corticosteroids for moderate to severe carditis, and long-term antibiotic prophylaxis to prevent recurrences.
rheumatic_feve for dentist 201`6--DR MAGDI SASIcardilogy
This document discusses rheumatic fever and rheumatic heart disease. Some key points:
- Rheumatic fever is an autoimmune disease that can develop after a streptococcal throat infection, causing inflammation in the heart, joints, brain, and skin. It is characterized by Jones criteria and symptoms include carditis, arthritis, chorea, and subcutaneous nodules.
- Rheumatic heart disease is scarring of the heart valves caused by repeated rheumatic fever infections. It can lead to stenosis or regurgitation of the valves. Symptoms depend on which valves are affected and include heart failure.
- Treatment involves antibiotics to prevent recurrent infections, anti-inflammatories, management
rheumatic_feve for dentist 201`6--DR MAGDI SASIcardilogy
This document discusses rheumatic fever and rheumatic heart disease. It begins by defining rheumatic fever as an inflammatory disease that occurs after a streptococcal infection, affecting the heart in 60% of cases. It then outlines the Jones criteria for diagnosing rheumatic fever based on evidence of prior streptococcal infection and symptoms. Treatment involves antibiotics, anti-inflammatories like aspirin, and long-term prevention with antibiotics. Rheumatic heart disease is a potential long-term complication if rheumatic fever causes permanent heart valve damage.
Giant cell arteritis (GCA) is a large vessel vasculitis that commonly affects the branches of the carotid artery and causes headaches, jaw claudication, and vision loss. The pathology involves granulomatous inflammation in the vessel walls. Diagnosis is based on temporal artery biopsy showing giant cells, but imaging such as ultrasound, CT, and PET can also provide supportive evidence of vessel inflammation. Treatment involves high-dose corticosteroids to reduce inflammation and prevent relapses and vision loss, with tapering over 2-5 years. Monitoring for complications like aortic aneurysms is also important given the vessel involvement.
The document discusses pathophysiology of mitral valve diseases. It begins with an overview of rheumatic fever and rheumatic heart disease as the leading cause of mitral stenosis worldwide. It then covers the anatomy of the mitral valve and describes the pathogenesis and hemodynamic consequences of mitral stenosis, including left atrial pressure overload, pulmonary hypertension, and symptoms like dyspnea. Late stages can involve left ventricular dysfunction. The document also briefly discusses etiology, classification and mechanisms of mitral regurgitation.
This document discusses the anatomy, types, diagnosis, and management of mesenteric ischemia. It begins with an overview of the mesenteric circulation anatomy and branches. It then covers the different types of mesenteric ischemia including arterial, venous, and non-occlusive forms. Diagnostic tools like ultrasound, CT angiography, and conventional angiography are summarized. Key findings on these imaging modalities that suggest mesenteric ischemia are provided. Management involves optimizing cardiac output, anti-coagulation, discontinuing precipitating medications, and administering vasodilators like papaverine or prostaglandins directly into the mesenteric arteries to resolve spasm. The goal is to increase bowel perfusion and prevent
This document discusses several types of vasculitis:
- Wegener's granulomatosis is a necrotizing vasculitis that commonly affects the lungs and kidneys. It is associated with cytoplasmic ANCA and affects small vessels.
- Microscopic polyangiitis is a pauci-immune vasculitis mainly involving small vessels. It commonly causes glomerulonephritis and pulmonary capillaritis.
- Churg-Strauss syndrome is characterized by asthma, eosinophilia, and vasculitis affecting multiple organ systems. It is associated with perinuclear ANCA.
Rheumatic fever and rheumatic heart disease are caused by an autoimmune reaction to untreated group A streptococcal infection. They commonly affect children aged 5-14 and can cause long-term heart damage through repeated episodes damaging the heart valves over time. Treatment involves antibiotics for the initial infection along with salicylates to reduce joint inflammation and fever. Lifestyle changes like a nutritious diet, stress management, and exercise can help with prevention and management of symptoms. Naturopathic treatments such as hydrotherapy, meditation, and yoga can also provide relief from joint pain and cardiac symptoms.
Rheumatic heart disease is a chronic condition that results from damage to the heart valves caused by rheumatic fever. Rheumatic fever is an inflammatory reaction that typically affects the heart, joints, brain and skin and is triggered by a prior streptococcal throat infection. It can cause scarring and deformity of the heart valves over time due to recurrent attacks. Treatment involves controlling streptococcal infections with antibiotics like penicillin to prevent recurrence of rheumatic fever and further heart damage. Patients are also at risk for developing valvular heart disease long-term.
Rheumatic fever- a multifactorial disease that follows GAS pharyngitis in a susceptible individual who lives under deprived social conditions, characterized by acute inflammation of the heart, joints, skin, subcutaneous tissue & CNS, that gives rise to typical clinical feature including Arthritis, Carditis, Chorea, Subcutaneous nodules & Erythema marginatum.
Latent period of 2-3 weeks following GAS pharangitis.
Destructive effects on heart valves leads to RHD with serious hemodynamic disturbances causing HF, stroke & infective endocarditis.
8 Surprising Reasons To Meditate 40 Minutes A Day That Can Change Your Life.pptxHolistified Wellness
We’re talking about Vedic Meditation, a form of meditation that has been around for at least 5,000 years. Back then, the people who lived in the Indus Valley, now known as India and Pakistan, practised meditation as a fundamental part of daily life. This knowledge that has given us yoga and Ayurveda, was known as Veda, hence the name Vedic. And though there are some written records, the practice has been passed down verbally from generation to generation.
These lecture slides, by Dr Sidra Arshad, offer a quick overview of the physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar lead (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
6. Describe the flow of current around the heart during the cardiac cycle
7. Discuss the placement and polarity of the leads of electrocardiograph
8. Describe the normal electrocardiograms recorded from the limb leads and explain the physiological basis of the different records that are obtained
9. Define mean electrical vector (axis) of the heart and give the normal range
10. Define the mean QRS vector
11. Describe the axes of leads (hexagonal reference system)
12. Comprehend the vectorial analysis of the normal ECG
13. Determine the mean electrical axis of the ventricular QRS and appreciate the mean axis deviation
14. Explain the concepts of current of injury, J point, and their significance
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. Chapter 3, Cardiology Explained, https://www.ncbi.nlm.nih.gov/books/NBK2214/
7. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
Cell Therapy Expansion and Challenges in Autoimmune DiseaseHealth Advances
There is increasing confidence that cell therapies will soon play a role in the treatment of autoimmune disorders, but the extent of this impact remains to be seen. Early readouts on autologous CAR-Ts in lupus are encouraging, but manufacturing and cost limitations are likely to restrict access to highly refractory patients. Allogeneic CAR-Ts have the potential to broaden access to earlier lines of treatment due to their inherent cost benefits, however they will need to demonstrate comparable or improved efficacy to established modalities.
In addition to infrastructure and capacity constraints, CAR-Ts face a very different risk-benefit dynamic in autoimmune compared to oncology, highlighting the need for tolerable therapies with low adverse event risk. CAR-NK and Treg-based therapies are also being developed in certain autoimmune disorders and may demonstrate favorable safety profiles. Several novel non-cell therapies such as bispecific antibodies, nanobodies, and RNAi drugs, may also offer future alternative competitive solutions with variable value propositions.
Widespread adoption of cell therapies will not only require strong efficacy and safety data, but also adapted pricing and access strategies. At oncology-based price points, CAR-Ts are unlikely to achieve broad market access in autoimmune disorders, with eligible patient populations that are potentially orders of magnitude greater than the number of currently addressable cancer patients. Developers have made strides towards reducing cell therapy COGS while improving manufacturing efficiency, but payors will inevitably restrict access until more sustainable pricing is achieved.
Despite these headwinds, industry leaders and investors remain confident that cell therapies are poised to address significant unmet need in patients suffering from autoimmune disorders. However, the extent of this impact on the treatment landscape remains to be seen, as the industry rapidly approaches an inflection point.
Does Over-Masturbation Contribute to Chronic Prostatitis.pptxwalterHu5
In some case, your chronic prostatitis may be related to over-masturbation. Generally, natural medicine Diuretic and Anti-inflammatory Pill can help mee get a cure.
Osteoporosis - Definition , Evaluation and Management .pdfJim Jacob Roy
Osteoporosis is an increasing cause of morbidity among the elderly.
In this document , a brief outline of osteoporosis is given , including the risk factors of osteoporosis fractures , the indications for testing bone mineral density and the management of osteoporosis
TEST BANK For Community Health Nursing A Canadian Perspective, 5th Edition by...Donc Test
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NVBDCP.pptx Nation vector borne disease control programSapna Thakur
NVBDCP was launched in 2003-2004 . Vector-Borne Disease: Disease that results from an infection transmitted to humans and other animals by blood-feeding arthropods, such as mosquitoes, ticks, and fleas. Examples of vector-borne diseases include Dengue fever, West Nile Virus, Lyme disease, and malaria.
- Video recording of this lecture in English language: https://youtu.be/kqbnxVAZs-0
- Video recording of this lecture in Arabic language: https://youtu.be/SINlygW1Mpc
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
- Link to NephroTube website: www.NephroTube.com
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share - Lions, tigers, AI and health misinformation, oh my!.pptxTina Purnat
• Pitfalls and pivots needed to use AI effectively in public health
• Evidence-based strategies to address health misinformation effectively
• Building trust with communities online and offline
• Equipping health professionals to address questions, concerns and health misinformation
• Assessing risk and mitigating harm from adverse health narratives in communities, health workforce and health system
Muktapishti is a traditional Ayurvedic preparation made from Shoditha Mukta (Purified Pearl), is believed to help regulate thyroid function and reduce symptoms of hyperthyroidism due to its cooling and balancing properties. Clinical evidence on its efficacy remains limited, necessitating further research to validate its therapeutic benefits.
3. HISTORY
In 1967- Tomisaku Kawasaki reports a case series of 50 patients
and concluded clinical criteria for diagnosis
1974- english language report of Kawasaki disease by Kawasaki
1976- first series of american patients was reported
1977- Landing and Larson establish Kawasaki disease and PAN
are pathologicallhy indistinguishable
1988- AAP endorses high dose IVIG and ASA as recommended
therapy for Kawasaki disease
4. INTRODUCTION
Kawasaki disease (KD) is an
acute, self-limited febrile
illness of unknown cause that
predominantly affects children
<5 years of age.
Vasculitis of medium sized
vessels
Aka- Mucocutaneous lymph node
syndrome or infantile PA N
MCC acquired heart disease in
developed nation
5. EPIDEMIOLOGY
The incidence was highest among Asians and Pacific Islanders (30.3
per 100 000 children <5 years of age)
Incidence in Japan- 210/100000 children <5 yrs
Male : female ratio- 1.5:1
Recurrence rate- 3-5% with highest in first 2 yrs after index episode
Genetic predisposition- 2.1 % risk in sibling
Most common in winter and spring in US , no such association in
tropics
A/W perinatal exposure like older maternal age, GBS infection,
hospitalisation in infancy
The case fatality rate is <0.1% in Japan
6. GENETICS
single-nucleotide polymorphisms in 6 genes or gene regions:
FcγR2a, caspase 3 (CASP3), human leukocyte antigen class II,
Bcell lymphoid kinase (BLK), inositol 1,4,5-trisphosphate
kinase-C (ITPKC), and CD40
Genetic variations in TGFb gene signalling pathway is a/w risk of
coronary anurysms
Risk and response to treatment is determined by genetic factors
7. PATHOGENESIS
Immunologically mediated event
Infection with a RNA virus which cause intracytoplasmic
inclusion bodies could be a cause
Activation of innate immune system is an early event
Fever cessation is associated with expansion of regulatory T cells
Self limited course and less risk of recurrence throws evidence to
activation of memory B cells
8. PATHOLOGY
Systemic inflammtion in all medium sized arteries
multiple organ and tissue involvement
hepatitis, gastroenteritis, meningitis, interstitial
pneuminia, pancarditis, pyuria, lymphadenopathy
9. PATHOLOGY
KD arteriopathy has 3 pathological process
Stage 1- necrotising arteritis with neutrophilic sequestration
first 2 weeks of fever onset
destroys arterial wall aneurysms
Stage 2- subacute or chronic vasculitis
infiltration of lymphocytes , plasma cells and eosinophils with
few macrophages
begins in 2 weeks , continues for months to yrs
Stage 3- luminal myofibroblastic proliferation (LMP)
medial smooth muscle cell–derived myofibroblastic process
progressive arterial stenosis
begins in first 2 weeks and persists for months to years
10. Pathological outcome depends on the severity of coronary artery
damage
Mildly dilated artery can regain its original structure
Giant saccular aneurysms may rupture or thrombus can canalise
or calcify
Fusiform aneurysms with preserved media cause LMP and later
stenose
Pericarditis and myocarditis results from acute or subacute
inflammation
11. DIAGNOSIS
Fever of atleast 5 days + any 4 out of the following 5 features
Erythema and cracking of lips, strawberry tongue, and/or erythema
of oral and pharyngeal mucosa
Bilateral bulbar conjunctival injection without exudate
Rash: maculopapular, diffuse erythroderma, or erythema
multiforme-like
Erythema and edema of the hands and feet in acute phase and/or
periungual desquamation in subacute phase
Cervical lymphadenopathy (≥1.5 cm diameter), usually unilateral
Patients who meet the case definition based on principal clinical
findings -complete KD/ typical KD
Patients who do not have sufficient principal clinical findings -
incomplete KD /atypical KD
12. Fever: high spiking with remittant nature
lasts for 1-3 weeks
resolves within 36 hrs of IVIG infusion- if not resistance
Extremity changes:
Acute: erythema of palms and soles and painful induration of
hands and feet
Chronic : desquamation of fingers and toes to involve palms and
soles
Beus lines appears over nails
Rash: diffuse maculopapular rash appears within 5days of fever
extensive and primarily involves trunk and extremities
sometimes groin
13. Conjunctivitis: bilateral bulbar non-exudative conjunctival
injection sparing the limbus
Oral mucosa: changes of the lips and oral cavity include (1)
erythema, dryness, fissuring, peeling, cracking, and bleeding
of the lips; (2) a “strawberry tongue,” with erythema and
prominent fungiform papillae; and (3) diffuse erythema of the
oropharyngeal mucosa.
Cervical lymphadenopathy:
Lymph node swelling is usually unilateral, ≥1.5 cm in diameter,
Isolated Cx lymphadenopathy- USG and CT differentiates it from
bacterial lymphadenitis
14. CVS- myocarditis, pericarditis, valve regurgitation and shock
coronary artery abnormaliities
aneurysms of non coronary medium sized vessels
aortic root enlargement and
peripheral gangrene
Resp: Pulmonary nodules
peribronchial and interstitial nodules
Musculoskeletal: arthritis and arthralgia
Gastrointestinal: diarrhea, vomiting ,pain abdomen
hepatitis, GB hydrops, pancreatitis
Nervous : irritability , SNHL, facial N palsy , aseptic meningitis
GUT: urethritis. Hydrocele
Others: desquamating rash in groin, retropharyngeal phlegmon,
erythema nd induration in BCG site
15. DD- Measles
viral infection –adeno
Streptococcal and staphylococcal toxin mediated disease
Drug hypersensitivity reactions
Systemic JIA
Rickettseal infections
leptospirosis
Incomplete KD:
• In any child (esp infant)with prolonged unexplained fever with
one or more principle clinical finding and compatile laboratory
and echocardiographic findings
16. When to suspect KD....
• Infants <6 months old with prolonged fever and irritability
• Infants with prolonged fever and unexplained aseptic
meningitis
• Infants or children with prolonged fever and unexplained or
culture-negative shock
• Infants or children with prolonged fever and cervical
lymphadenitis unresponsive to antibiotic therapy
• Infants or children with prolonged fever and retropharyngeal
or parapharyngeal phlegmon unresponsive to antibiotic
therapy
17.
18. Laboratory tests:
• Leucocytosis with granulocytosis
• Anemia is normochromic and normocytic
• ESR andCRP- elevated
CRP normalizes more quickly than the ESR
ESR increases after IVIG thearpy
Decreased ESR with severe clinical symptoms s/o DIC
• Thrombocytosis – seen in 2 nd week and peaks in 3rd week and
resolves by 4-6 th week
thrombocytopenia in acute phase DIC
• Mild to moderate elevations in serum transaminases
• Hypoalbuminemia
• Urinanalysis- sterile pyuria
• CSF study- pleocytosis
19. CVS findings
CLINICAL:
The pericardium, myocardium, endocardium including valves, and the
coronary arteries all may be inflamed
Tachycardia , hyperdynamic precordium, innocent systolic murmur,
gallop rhythm.
Pericardial rub in case of tamponade
Valvar dysfunction occurs in ≈25% of patients mc mitral valve
PSM due to MR and diastolic murmur due to AR may be heard
ECG:
SA and AV node functional abnormalities
prolonged PR interval
nonspecific ST and T-wave changes
low voltage if there is myocardial or pericardial involvement
20. CV COMPLICATIONS
CV collapse:
Seen in 5% of cases
Septic shock must be ruled out
Children with shock presentation appear to be at higher risk of
IVIG resistance, coronary artery abnormalities, MR, and
prolonged myocardial dysfunction.
Myocardial dysfunction:
Develops early and transient
Improves rapidly as the inflammatory process results from
interstitial edema and inflammation and only rarely from
myocardial cell necrosis
Asso with ventricular ectopy
21. Valvular and aortic abnormalities:
MR is seen in 25% of KD
Asso with pancarditis
Mild to moderate in severity and is transient
AR is less common (<1%)
AR seen in asso with aortic root dialatation
Aotic root dilatation occurs in 10% of cases
Coronary artery abnormalities:
Specific diagnostic criteria for incomplete KD
Ranges from dilatation to aneurysms, proximal to distal
Majority have dilatation only with z score of 2-2.5, resolve sin 4-
8 weeks
Giant aneurysms can cause ischemia due to thrombosis or rupture
Other arterial involvement:
22. EVALUATION OF CARDIOVASCULAR
ABNORMALITIES
Echocardiography:
Noninvasive and has a high sensitivity and specificity
Initial echo must be performed at the time of diagnosis
Initial study is always normal in 1st week and act as baseline for
further follow up
Using high frequency transducer
In the order of frequency:
proximal LAD and proximal RCA > LMCA >left circumflex >
distal RCA > junction between the RCA and posterior
descending coronary artery
23. Qualitative and quantitative assessment:
Evaluation of coronaries – with internal diameter
Exclude branching points
Identify the number and location of thrombus
Types – saccular, fusiform and ectatic
Japanese guidelines- classify according to absolute and relative
internal diameter
< 5 yrs >5 yrs
Small <4mm 1.5 times
Med >4 , </= 8mm 1.5 to 4 times
Large >8mm >4 times
24. Defining abnormality-
Z score of >2.5 in one coronary is normal in 0.6 % and >3 in
0.1%
Z score more than 2.5 in two coronaries (LAD and RCA)not
normal
Anatomical variation of LMCA exist
Z-Score Classification :
1. No involvement: Always <2
2. Dilation only: 2 to <2.5; or if initially <2, a decrease in Z
score during follow-up ≥1
3. Small aneurysm: ≥2.5 to <5
4. Medium aneurysm: ≥5 to <10, and absolute dimension <8 mm
5. Large or giant aneurysm: ≥10, or absolute dimension ≥8 mm
25. Assess ventricular function and ejection fraction
Assess systolic and diastolic dimensions of ventricle and regional wall
motion abnormalities
Assessment of aortic root:
Root dilatation >2 z score in 10% KD
Look for pericardial effusion and valvular regurgitation
For uncomplicated patients, echo should be repeated both within 1 to 2
weeks and 4 to 6 weeks after treatment
For evolving CAD , echo must be repeated twice per week till its size
stops progressing to contain a thrombus
With thrombosis echo is indicated once weekly for first 45 days and
monthly till 3 months
Other imaging modalities:
TEE
Invasive angiography
CTA
CMRI
26. TREATMENT OF ACUTE ILLNESS
From onset of acute illness to resolution of acute systemic
inflammation when coronaries stop expanding
Single high dose IVIG plus ASA
Patient selection:
With complete KD and incomplete KD
IVIG should be given once diagnosis is made, within 10 days of
onset of illness
IVIG should be given to children presenting after 10th day of
illness if
a) persistent unexplained fever
b) elevated CRP or ESR
c) coronary dimensions z score more than 2.5
d) recurrent KD
27. IVIG:
modulation of cytokine production,
neutralization of toxins or other pathogenic agents
augmentation of regulatory T-cell activity
suppression of antibody synthesis and provision of
antiidiotypic antibodies
Lowers the incidence of coronary artery abnormalities
2 g/kg as a single infusion, usually given over 10 to 12 hours,
together with ASA
S/E: hemolytic anemia, aseptic meningitis (without any sequela)
defer all live vaccines for next 11 months of administartion
28. ASA:
High dose act as antiinflammatory and low dose act as
antiplatelet
Does not lower the incidence of CA anbormality
Dose: 80-100mg/kg/d every 6 th hourly
High dose is given for 48-72hrs of afebrile period or 14 days of
illness
Low-dose ASA (3 to 5 mg/kg/d) is given after that and continued
until the patient has no evidence of coronary changes by 6 to 8
weeks after onset of illness
If they develop CA abnormality it is further continued
S/E- Reye syndrome- develops influenza or varicella while on
high dose ASA
Alternative antiplatelet agent must be given and immunise the
child
29. ADJUNCT THERAPY
For those at the risk of coronary artery abnormality
Corticosteroid:
Lower incidence of coronary artery abnormalities
Lower risk of retreatment
Rapid resolution of fever and more rapid decrease in CRP
levels
Lower risk of IVIG resistance
Regimen: IVIG (2 g/kg for 1 day) + ASA (30 mg/kg/d) plus
intravenous prednisolone (2 mg/kg/d) for 5 days followed by
an oral taper over weeks
30. Infliximab:
Anti TNF alpha monoclonal antibody
Halts inflammation in resistant KD
Decrease in incidence of IVIG resistance
Rapid reduction in inflammatory parameters
Decrease the rate of IVIG reactions
Dose: 5mg/kg/dose iv over 2 hrs
Etanercept: (soluble TNF receptor)
Administer subcutaneously after IVIG and repeat at 1 and 2
weeks later
31. IVIG Resistance
Recrudescent or persistent fever at least 36 hours after the end of the
IVIG infusion
Incidence- 10-20%
Polymorphism in Fc gama receptor
Treatment of IVIG resistance:
1) IVIG: Second infusion- 2 g/kg/dose
2) IVIG + prednisolone: IVIG 2 g/kg IV + pred 2 mg/kg/dose IV
divided every 8 h until afebrile, then orally until CRP normalise,
then taper over 2–3 wk
3) Cyclosporine- IV: 3 mg/kg/d, divided every 12 h PO: 4–8 mg/kg/d
divided every 12 h
4) Anakinra- 2–6 mg/kg /d given by subcutaneous injection
5) Cyclophosphamide - 2 mg/kg/dose
6) Plasma exchange
32. Cyclospsorine:
Specific inhibitor of calcineurin
NFAT-calcineurin calcium signaling pathway contribute to
inflammatory action in KD
Dose- IV 3 mg/kg/d, divided every 12 h, PO: 4–8 mg/kg/d
divided every 12 h
Once the patient is afebrile + clinically improving + CRP is ≤1.0
mg/dL, or after 2 weeks of therapy, the dose can be tapered by
10% of the initial dose every 3 days and discontinued when
the dose has reached 1 mg/kg/d
Anakinra- IL-1 antagonist
Plasma exchange- in case medical therapy fails
33. Treatment of acute myocardial dysfunction:
KDSS results from interstitial edema and cellular infiltration
KDSS – hypotension and shock requiring the initiation of volume
expanders, the infusion of vasoactive agents, or transfer to
intensive care units
Mechanism
• Release of endogenous molecules that mediate a decrease in
peripheral vascular resistance (distributive)
• myocardial dysfunction from myocarditis with or without
myocardial ischemia(cardiogenic)
• capillary leakage
Treatment: fluids + ionotropes + IVIG
34. Treatment and prevention of coronary artery aneurysm
Thrombocytosis , increased platelet adhesion, inflammation, and
endothelial dysfunction, together with abnormal flow
conditions through areas of severe dilation.
For small aneurysm: monotherapy with low-dose ASA therapy is
sufficient for prophylaxis of thrombosis.
For mod aneurysm: ASA therapy combined with a
thienopyridine (eg, clopidogrel)
For giant aneurysm: “triple therapy” with ASA, a second
antiplatelet agent, and anticoagulation with warfarin (INR- 2 to
3)or LMWH
Anticoagulation in infants with LMWH
Transition from LMWH to warfarin once aneurysms stopped
expanding and the patient is stable
35. Treatment :
Goals: reestablishing coronary artery patency and flow, salvaging
myocardium, and improving survival
By thrombolytic therapy or mechanical restoration of coronary
artery flow by cardiac catheterisation
Thrombolysis by tPA - in infants and children
Dose- 0.5mg/kg/hr over 6hrs
Along with low dose aspirin and low dose heparin
Monitor coagulation parameters
Maintain fibrinogen level more than 100mg/dl
Maintain platelet count more than 50000
After tPA increase heparin dose and assess thrombus by echo
If rebound increase in thrombus size or same size of thrombus ,
then low dose thrombolytic with abciximab
36. Coronary artery events (thrombosis, stenosis, intervention, MI,
death)
Late development or increases in size of aneurysms have been
reported in case reports.
Late-onset valvulitis of the mitral and aortic valves (rare)and
may require valve replacement.
MR can occur after the acute stage from myocardial ischemia.
AR in KD is usually associated with aortic root dilation and
apparent early in the course of the disease
Long-term myocardial dysfunction, resulting from primary
myocardial insult at the time of acute KD
Premature ventricular contractions and ventricular tachycardia
are common
37. LONGTERM MANAGEMENT
Begins at the end of acute illness ie, 4-6 weeks after fever onset
Goal: prevent thrombosis and MI
Medically – thromboprophylaxis and surveillance for coronary
artery stenosis and obstruction
Intervention – coronary revascularisation by transcatheter or
coronary bypass graft or cardiac transplantation
39. No involvement:
Follow up to 1yr
Low dose ASA for upto 4-6wks and discontinue later
Check BP, lipid profile, dietary assessment atleast once after 1 yr
No additional medical therapy
No restriction on physical activity
Dialtation only(z score>/= 2 < 2.5 or decrease in z score during
follow up)
Lumen dimension returned to normal by 4-6 wks F/U upto 1yr
Dimension not returned to normal follow up to 12 months
If dilatation present at 1 yr continue follow up every 2-5 yrs
Low dose ASA for upto 4-6 wks and discontinue later
40. No restriction on physical activity
Counseling regarding healthy lifestyle and activity promotion at
every visit
Small aneurysm z score >/=2.5 <5
a) Current or persistant
Patient should be seen at 4-6 weeks after onset, then follow up at
6 months and 1 yr and annually later
Assess for inducible MI at every 2-3 yrs if symptoms o f MI or
dysfunction present
Imaging with angio and CT every 3-5 yrs
Check BP, lipid profile, dietary assessment atleast once after 1yr
Low dose ASA or clopidogrel can be given
Empirical statin therapy can be considered
No restriction on physical activity
41. b) Regression to normal Z score or dilatation
Regular follow up every 1-3 yrs
Assess for inducible MI every 2-3 yrs if symptomatic
Additional imaging if evidence for inducible MI
Medium aneurysms
a) Current or persistent
Review at 4-6 weeks, then 3 Mo, 6Mo and at 1 yr. Follow up
assessment every 6-12 months later
Assess for inducible myocardial ischemia every 1-3 yr
Further imaging with angio every 2-5 yrs
Counsel regarding healthy life style and activity promotion
Medications:
Low dose ASA
42. Empirical statin therapy
Use of alternative antiplatelet agent can be consisdered
Dual antiplatelet therapy can be considered
Perform FLP ,BMI dietary assessment annually
Use of anticoagulant is not recommended
L/F coronary artery characteristics to intensify thromboprophylaxis
Restricted physical activity- inducible MI testing before competitive
sports and also for those on dual therapy
Discourage the use of OCPs as contraceptive methods
b) Regression to small aneurysm:
Annual follow up assessment
Assess for inducible MI every 2-3 yrs
Additional imaging every 3-5 yrs
Medications:
43. Low dose ASA
Low dose statin therapy
Dual anti platelet therapy may be considered
Normal activity no restriction, competitive sport after MI test
c) Regression to normal z score or dilatation
Follow up assessment every 1-2 yrs
Assess for inducible MI every 2-4 yrs and no further imaging
CV risk assessment every 2 yrs
Medication : Statins, ASA, alternative antiplatelet and no
additional antiplatelet
No limitation in daily activity, competitive sports after inducible
MI test
44. Large Aneurysms:
a)Current or persistent:
Assess patients at 1, 2, 3, 6, 9, and 12 months after the episode of
acute KD in the first year and every 3 to 6 months thereafter
Assess for inducible MI every 6-12 months
Further imaging every 1-5 yrs
CV risk assessment every 6-12 months
Medications:
Empirical statin and beta blocker
Low dose ASA
Additional antiplatelet therapy
Warfarin to be considered to achieve INR 2-3/ LMWH as an
alternative
45. b)Regression to medium aneurysm:
Assess every 6-12 months
Assess for inducible MI annually
Medications:
Use of statin and beta blocker is considered
Low dose aspirin
Anticoagulation is not indicated
c) Regression to small aneurysm or normal size:
Assessment is same
Medications:
Low dose aspirin and beta blocker
Beta blocker is not indicated if size normalise
No additional antiplatelet or anticoagulant
46. Revascularisation
Revascularization should be avoided in KD patients in the
acute/subacute phase of the illness with STEMI attributable to
acute thrombotic occlusion of an aneurysm
Adult patients with remote history of KD presenting with STEMI
should be referred emergently for coronary angiography
Revascularization should be performed in KD patients
a) with stable angina and high-risk coronary anatomy including
left main CAD, multivessel coronary disease with reduction in
LV function, multivessel coronary disease with diabetes
mellitus
b)non–ST-segment elevation and coronary anatomy amenable to
revascularization on coronary angiography
47. c) for patients with stable angina and symptoms refractory to
maximal medical therapy
d) patients with silent ischemia and ischemia involving >10% of
LV mass may be considered
CABG vs PCI
CABG is preferred to PCI in KD patients with left main CAD,
multivessel CAD with reduced LV function, multivessel CAD
with lesions not amenable to PCI, and multivessel CAD in
diabetic patients
PCI is preferred in patients with single-vessel or focal multivessel
disease amenable to PCI
CABG is preferred to PCI in older children and adults with KD
and multivessel involvement
48. Cardiac transplantation:
It is reasonable to consider cardiac transplantation for patients
with severe, irreversible myocardial dysfunction and coronary
artery lesions for which interventional catheterization
procedures or CABG are not feasible