Tag Archives: Cardiovascular Disorders

Sepsis

 

Alternative Names: Systemic inflammatory response syndrome (SIRS),blood poisoning or septicaemia.

Definition:
Sepsis is a bacterial infection of the blood.It is a severe illness in which the bloodstream is overwhelmed by bacteria.While sepsis can happen to anyone, it’s most common and most dangerous in people who are elderly or who have weakened immune systems.

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Sepsis occurs when chemicals released into the bloodstream to fight the infection trigger inflammation throughout the body. This inflammation creates microscopic blood clots that can block nutrients and oxygen from reaching organs, causing them to fail. If sepsis progresses to septic shock, blood pressure drops dramatically and the person may die.

Early treatment of sepsis, usually with antibiotics and large amounts of intravenous fluids, improves chances for survival.

Symptoms:
In addition to symptoms related to the provoking infection, sepsis is characterized by presence of acute inflammation present throughout the entire body, and is, therefore, frequently associated with fever and elevated white blood cell count (leukocytosis) or low white blood cell count and lower-than-average temperature, and vomiting. The modern concept of sepsis is that the host’s immune response to the infection causes most of the symptoms of sepsis, resulting in hemodynamic consequences and damage to organs. This host response has been termed systemic inflammatory response syndrome (SIRS) and is characterized by an elevated heart rate (above 90 beats per minute), high respiratory rate (above 20 breaths per minute or a partial pressure of carbon dioxide in the blood of less than 32), abnormal white blood cell count (above 12,000, lower than 4,000, or greater than 10% band forms) and elevated or lowered body temperature, i.e. under 36 °C (97 °F) or over 38 °C (100 °F). Sepsis is differentiated from SIRS by the presence of a known or suspected pathogen. For example SIRS and a positive blood culture for a pathogen indicates the presence of sepsis. However, in many cases of sepsis no specific pathogen is identified.

This immunological response causes widespread activation of acute-phase proteins, affecting the complement system and the coagulation pathways, which then cause damage to the vasculature as well as to the organs. Various neuroendocrine counter-regulatory systems are then activated as well, often compounding the problem. Even with immediate and aggressive treatment, this may progress to multiple organ dysfunction syndrome and eventually death.

Causes:
Sepsis is often a complication of another infection, such as of the lungs or kidneys, and occurs when the bacteria escape that part of the body and get into the bloodstream.

This bacteria can also come from burns, infected wounds, boils and tooth abscesses. Sometimes it isn’t obvious how it has got into your blood.

Anyone can develop sepsis. The people most at risk are those with weakened immune systems, because of an existing illness, for example, or medication.

Older people, children and intravenous drug users are also more susceptible.

In children, sepsis may accompany infection of the bone (osteomyelitis). In hospitalized patients, common sites of infection include intravenous lines, surgical wounds, surgical drains, and sites of skin breakdown known as bedsores (decubitus ulcers).

Risk Factors:
Sepsis is more common and more dangerous in people who:

*Are very young or very old
*Have compromised immune systems
*Are already very sick, often in a hospital’s intensive care unit
*Have invasive devices, such as intravenous catheters or breathing tubes

Complication:
As sepsis worsens, blood flow to vital organs, such as your brain, heart and kidneys, becomes impaired. Sepsis can also cause blood clots to form in your organs and in your arms, legs, fingers and toes — leading to varying degrees of organ failure and tissue death (gangrene).

Most people recover from mild sepsis, but the mortality rate for severe sepsis or septic shock is close to 50 percent.

Diagnosis:
The infection is often confirmed by a blood test. However, a blood test may not reveal infection in people who have been receiving antibiotics.

Other tests that may be done include:
•Blood gases
•Kidney function tests
•Platelet count
•White blood cell count
•Blood differential
•Fibrin degradation products
•Peripheral smear

Treatment;
Early, aggressive treatment boosts your chances of surviving sepsis. People with severe sepsis require close monitoring and treatment in a hospital intensive care unit. If one has severe sepsis or septic shock, lifesaving measures may be needed to stabilize breathing and heart function.

Medications
A number of different types of medications are used in treating sepsis. They include:

*Antibiotics. Treatment with antibiotics begins immediately — even before the infectious agent is identified. Initially you’ll receive broad-spectrum antibiotics, which are effective against a variety of bacteria. The antibiotics are administered intravenously (IV). After learning the results of blood tests, your doctor may switch to a different antibiotic that’s more appropriate against the particular bacteria causing the infection.

*Vasopressors. If your blood pressure remains too low even after receiving intravenous fluids, you may be given a vasopressor medication, which constricts blood vessels and helps to increase blood pressure.

*Others. Other medications you may receive include low doses of corticosteroids, insulin to help maintain stable blood sugar levels, drugs that modify the immune system responses, and painkillers or sedatives.Therapy

People with severe sepsis usually receive supportive care including oxygen and large amounts of intravenous fluids. Depending on your condition, you may need to have a machine help you breathe or dialysis for kidney failure.

Surgery
Surgery may be needed to remove sources of infection, such as collections of pus (abscesses).

Prognosis:
This section requires expansion.

Prognosis can be estimated with the Mortality in Emergency Department Sepsis (MEDS) score.  Approximately 20–35% of patients with severe sepsis and 40–60% of patients with septic shock die within 30 days. Others die within the ensuing 6 months. Late deaths often result from poorly controlled infection, immunosuppression, complications of intensive care, failure of multiple organs, or the patient’s underlying disease.

Prognostic stratification systems such as APACHE II indicate that factoring in the patient’s age, underlying condition, and various physiologic variables can yield estimates of the risk of dying of severe sepsis. Of the individual covariates, the severity of underlying disease most strongly influences the risk of death. Septic shock is also a strong predictor of short- and long-term mortality. Case-fatality rates are similar for culture-positive and culture-negative severe sepsis.

Some patients may experience severe long-term cognitive decline following an episode of severe sepsis, but the absence of baseline neuropsychological data in most sepsis patients makes the incidence of this difficult to quantify or to study. A preliminary study of nine patients with septic shock showed abnormalities in seven patients by MRI.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.
Resources:
http://www.mayoclinic.com/health/sepsis/DS01004
http://www.nlm.nih.gov/medlineplus/ency/article/000666.htm
http://en.wikipedia.org/wiki/Sepsis
http://www.bbc.co.uk/health/physical_health/conditions/sepsis.shtml
http://www.humenhealth.com/sepsis/sepsis.asp
http://images.emedicinehealth.com/images/4453/4453-4482-12996-21147.jpg

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Pericarditis

Definition:
The pericardium is a thin, two-layered, fluid-filled sac that covers the outer surface of the heart. It shields the heart from infection or malignancy and contains the heart in the chest wall. It also prevents the heart from over-expanding when blood volume increases, which keeps the heart functioning efficiently.
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Pericarditis is an inflammation of the pericardium (the fibrous sac surrounding the heart). A characteristic chest pain is often present.

It can be caused by a variety of causes including viral infections of the pericardium, idiopathic causes, uremic pericarditis, bacterial infections of the precardium (for i.e. Mycobacterium tuberculosis), post-infarct pericarditis (pericarditis due to heart attack), or Dressler’s pericarditis.

If you could see and touch it, the membrane around the heart would look red and swollen, like the skin around a cut that becomes inflamed. Sometimes excess fluid develops in the space between the pericardial layers and causes a pericardial effusion (buildup of excess fluid around the heart).

Classification:
Pericarditis can be classified according to the composition of the inflammatory exudate or in other words the composition of the fluid that accumulates around the heart.

Types include:

*serous
*purulent
*fibrinous
*caseous
*hemorrhagic
*Post infarction

Acute vs. chronic:
Depending on the time of presentation and duration, pericarditis is divided into “acute” and “chronic” forms. Acute pericarditis is more common than chronic pericarditis, and can occur as a complication of infections, immunologic conditions, or even as a result of a heart attack (myocardial infarction). Chronic pericarditis however is less common, a form of which is constrictive pericarditis.

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The following is the clinical classification of acute vs. chronic:
Clinically: Acute (<6 weeks), Subacute (6 weeks to 6 months) and Chronic (>6 months)

 

Symptoms:
Acute pericarditis usually lasts less than a few weeks. Chronic pericarditis usually lasts six months or longer.

If you have acute pericarditis, the most common symptom is sharp, stabbing chest pain behind the breastbone or in the left side of your chest. However, some people with acute pericarditis describe their chest pain as dull, achy or pressure-like instead, and of varying intensity.

The pain of acute pericarditis may travel into your left shoulder and neck. It often intensifies when you lie down or inhale deeply. Coughing, taking a deep breath or swallowing food also may make the pain worse. Sitting up and leaning forward can often ease the pain. At times, it may be difficult to distinguish pericardial pain from the pain that occurs with a heart attack.

Chronic pericarditis is usually associated with chronic inflammation and may result in fluid around the heart (pericardial effusion). The most common symptom of chronic pericarditis is chest pain.

Depending on the type, signs and symptoms of pericarditis may include some or all of the following:

*Sharp, piercing chest pain over the center or left side of your chest, which is worse when taking a deep breath and relieved by sitting forwards – many people think they’re having a heart attack

*Shortness of breath when reclining

*Low-grade fever

*An overall sense of weakness, fatigue or feeling sick

*Dry cough and fatigue

*Fever

*Abdominal or leg swelling

*Pain in the shoulders and neck

Causes:
Under normal circumstances, the two-layered pericardial sac that surrounds your heart contains a small amount of lubricating fluid. In pericarditis, the sac becomes inflamed and the resulting friction from the inflamed sac leads to chest pain.

In some cases the amount of fluid contained in the pericardial sac may increase, causing a pericardial effusion.

The cause of pericarditis is often hard to determine. In most cases doctors are either unable to determine a cause (idiopathic) or suspect a viral infection.

Pericarditis can also develop shortly after a major heart attack, due to the irritation of the underlying damaged heart muscle. In addition, a delayed form of pericarditis may occur weeks after a heart attack or heart surgery because of antibody formation. This delayed pericarditis is known as Dressler’s syndrome. Many experts believe Dressler’s syndrome is due to an autoimmune response, a mistaken inflammatory response by the body to its own tissues — in this case, the heart and pericardium.

Other causes of pericarditis include:

*Systemic inflammatory disorders. These may include lupus and rheumatoid arthritis.

*Trauma. Injury to your heart or chest may occur as a result of a motor vehicle or other accident.

*Other health disorders. These may include kidney failure, AIDS, tuberculosis and cancer.

*Certain medications. Some medications can cause pericarditis, although this is unusual.

Complication:
Constrictive pericarditis is a severe form of chronic pericarditis in which the inflamed layers of the pericardium stiffen, develop scar tissue, thicken and stick together. The thick, rigid pericardium constricts the heart’s normal movement so that it cannot expand normally as it fills with blood. As a result, the heart chambers don’t fill up with enough blood. The blood then backs up behind the heart, causing symptoms of heart failure, including shortness of breath, swelling of the legs and feet, water retention and disturbances in the heart’s normal rhythm. These symptoms should improve when the constrictive pericarditis is treated.

Constrictive pericarditis often can be treated with a diuretic, such as furosemide, to treat the fluid retention. If you develop a heart rhythm problem, you may need to take a medication to treat the irregular rhythm for as long as the constrictive pericarditis lasts or until your heart rhythm returns to normal. When none of these treatments is effective, pericardiectomy may be needed to surgically remove the stiffened pericardium.

Pericardial Effusion
When an excess of fluid builds up in the space between the pericardium, it can cause a condition known as pericardial effusion. Rapid fluid accumulation in the pericardium can cause cardiac tamponade, a severe compression of the heart that impairs its ability to function. Cardiac tamponade resulting from a pericardial effusion can be life-threatening and is a medical emergency requiring emergent drainage of the fluid with a catheter.

 

 

Diagnosis:
The classic sign of pericarditis is a friction rub auscultated on the cardiovascular examination usually on the lower left sternal border. Other physical signs include a patient in distress, positional chest pain, diaphoresis (excessive sweating), and possibility of heart failure in form of precardial tamponade causing pulsus paradoxus, and the Beck’s triad of hypotension (due to decreased cardiac output), distant (muffled) heart sounds, and JVD (jugular vein distention).

When listening to the heart with a stethoscope, the health care provider can hear a sound called a pericardial rub. The heart sounds may be muffled or distant. There may be other signs of fluid in the pericardium (pericardial effusion).

If the disorder is severe, there may be:

•Crackles in the lungs
•Decreased breath sounds
•Other signs of fluid in the space around the lungs (pleural effusion)

.
If fluid has built up in the pericardial sac, it may show on:

•Chest MRI scan
•Chest x-ray
•ECG
•Echocardiogram
•Heart MRI or heart CT scan
•Radionuclide scanning
These tests show:

•Enlargement of the heart
•Signs of inflammation
•Scarring and contracture of the pericardium (constrictive pericarditis)
Other findings vary depending on the cause of pericarditis.

To rule out heart attack, the health care provider may order serial cardiac marker levels (CPK-MB and troponin I). Other laboratory tests may include:

•Blood culture
•CBC
•C-reactive protein
•Erythrocyte sedimentation rate (ESR)
•HIV serology
•Pericardiocentesis, with chemical analysis and pericardial fluid culture
•Tuberculin skin test

Treatment:
The cause of pericarditis must be identified, if possible.

Medications include:

•Analgesics for pain
•Antibiotics for bacterial pericarditis
•Antifungal medications for fungal pericarditis
•Aspirin or a nonsteroidal anti-inflammatory drug (NSAID) such as ibuprofen for inflammation of the pericardium
•Corticosteroids such as prednisone (in some patients)
•Colchicine
If the buildup of fluid in the pericardium makes the heart function poorly or produces cardiac tamponade, it is necessary to drain the fluid from the sac. This procedure, called pericardiocentesis, may be done using an echocardiography-guided needle or minor surgery.

If the pericarditis is chronic, recurrent, or causes constrictive pericarditis, cutting or removing part of the pericardium may be recommended.

Prognosis:
Pericarditis can range from mild cases that get better on their own to life-threatening cases. The condition can be complicated by significant fluid buildup around the heart and poor heart function.

The outcome is good if the disorder is treated promptly. Most people recover in 2 weeks to 3 months. However, pericarditis may come back.

Prevention:
Many cases are not preventable.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://www.nlm.nih.gov/medlineplus/ency/article/000182.htm
http://www.mayoclinic.com/health/pericarditis/DS00505
http://en.wikipedia.org/wiki/Pericarditis
http://my.clevelandclinic.org/heart/disorders/other/pericarditis.aspx

http://www.nlm.nih.gov/medlineplus/ency/imagepages/18080.htm

http://fromyourdoctor.com/topic.do?title=Pericarditis&t=8158

Lifestyle for a Healthy Heart

Heart disease may be inherited, but often it’s the result of lifestyle. Changing eating, exercise and smoking habits can play a significant part in prevention.

The following risk factors can cause heart disease. While there are some you can do little or nothing about, there are others that are worth addressing to make sure you keep a healthy heart:
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Age
Four out of five people who die from coronary heart disease are aged 65 or older.

Gender
Men are more at risk of heart disease than women and have heart attacks earlier in life. However, death rates from heart disease and stroke for women are twice as high as those for all forms of cancer.

The risk for women increases as they approach menopause and continues to rise as they get older, possibly because of the loss of oestrogen, the natural hormone.

Family history
Children of parents with heart disease are more likely to suffer from the disease themselves. Some races, such as Afro-Caribbeans, are more prone to coronary heart disease and stroke than others.

Smoking
Smokers are twice as likely to suffer heart attacks as non-smokers and are more likely to die as a result. Smoking is also linked to increased risk of stroke.

The nicotine and carbon monoxide in tobacco smoke damages the cardiovascular system. Passive smoking may also be a danger.

Women who smoke and take the oral contraceptive pill are at high risk of heart disease and stroke.

Alcohol
Drinking an average of more than one drink a day for women or more than two drinks a day for men increases the risk of heart disease and stroke because of the effect on blood pressure, weight and levels of triglycerides, a type of fat carried in the blood.

Binge drinking is particularly dangerous.

Drug abuse
The use of certain drugs, particularly cocaine and those taken intravenously, has been linked to heart disease and stroke.

Cocaine can cause abnormal heartbeat, which can be fatal, while heroin and opiates can cause lung failure. Injecting drugs can cause an infection of the heart or blood vessels.

Cholesterol
The higher the blood cholesterol level, the higher the risk of coronary heart disease, particularly if it’s combined with any of the other risk factors.

Diet is one cause of high cholesterol; others are age, gender and family history.

Blood pressure
High blood pressure increases the heart’s workload, causing it to enlarge and weaken over time. When combined with obesity, smoking, high cholesterol or diabetes, the risk increases several times.

High blood pressure can be a problem in women who are pregnant or are taking high-dose types of oral contraceptive pill.

Physical inactivity
Failure to exercise is a cause of coronary heart disease as physical activity helps control cholesterol levels, diabetes and, in some cases, can help lower blood pressure.

Obesity
People who are overweight are more likely to develop heart disease and stroke, even if they have none of the other risk factors. Excess weight causes extra strain on the heart, influences blood pressure, cholesterol and levels of other blood fats – including triglycerides – and increases the risk of developing diabetes.

 

Diabetes
The condition seriously increases the risk of developing cardiovascular disease, even if glucose levels are under control. More than 80 per cent of people with diabetes die of some form of heart or blood vessel disease.

Previous medical history
People who have had a previous heart attack or stroke are more likely than others to suffer further events.

Stress
Some links have been made between stress and coronary artery disease. This could be because it encourages people to eat more, start smoking or smoke more than they would otherwise have done.

Source:BBC Health

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Kawasaki disease

Alternative Names: Kawasaki syndrome, lymph node syndrome and mucocutaneous lymph node syndrome

Definition:
Kawasaki disease (KD) is an autoimmune disease that manifests as a systemic necrotizing medium-sized vessel vasculitis and is largely seen in children under five years of age. It affects many organ systems, mainly those including the blood vessels, skin, mucous membranes and lymph nodes; however, its most serious effect is on the heart where it can cause severe coronary artery aneurysms in untreated children. Without treatment, mortality may approach 1%, usually within six weeks of onset. With treatment, the mortality rate is less than 0.01% in the U.S. There is often a pre-existing viral infection that may play a role in its pathogenesis. The conjunctivae and oral mucosa, along with the epidermis (skin), become erythematous (red and inflamed). Edema is often seen in the hands and feet and one or both of the cervical lymph nodes are often enlarged. Also, a remittant fever, often 40? (104°F) or higher, is characteristic of the acute phase of the disease. In untreated children, the febrile period lasts on average approximately 10 days, but may range from five to 25 days. The disorder was first described in 1967 by Dr. Tomisaku Kawasaki in Japan.

Kawasaki disease affects boys more than girls with people of Asian ethnicity, particularly Japanese and Korean most susceptible as well as people of Afro-Caribbean ethnicity. The disease was rare in Caucasians until the last few decades and incidence rate fluctuates from country to country.

By far the highest incidence of Kawasaki disease occurs in Japan (175 per 100,000).

However its incidence in the United States is increasing. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than five years of age. Approximately 2,000-4,000 cases are identified in the United States each year.

In the United Kingdom, estimates of incidence rate vary because of the rarity of Kawasaki disease. However Kawasaki disease is believed to affect fewer than 1 in every 25,000 people.  Incidence of the disease doubled from 1991 to 2000 however, with 4 cases in per 100,000 children in 1991 compared with a rise of 8 cases per 100,000 in 2000.

Symptoms:
Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal treatment with paracetamol (acetaminophen) or ibuprofen.The fever may persist steadily for up to two weeks and is normally accompanied by irritability. Affected children develop red eyes because of non-suppurative conjunctivitis, iritis and bilateral anterior uveitis  Inflammation of the mucous membranes in the mouth, along with erythema (redness), edema (swelling) with fissures (cracks in the lip surface), desquamation (peeling) and exsudation of the lips are also evident. The oropharynx mucosa has enanthema and the tongue maintains an unusual red appearance termed “strawberry tongue” (marked erythema with prominent gustative papillae). Keratic precipitates (detectable by a slit lamp but usually too small to be seen by the unaided eye), and swollen lymph nodes may also be present and can be the first manifestation of the disease. Rashes occur early in the disease, and the cutaneous rash observed in patients with KD is non-specific, polymorphic, non-itchy and normally observed up to the fifth day of fever. Cutaneous exanthema may comprise macular-papular erythematous and fissure lesions, the most common type, in addition to urticariform type rash, purpuric, multiform-like erythema. and peeling of the skin in the genital area, hands, and feet (especially around the nails and on the palms and soles) may occur in later phases. Some of these symptoms may come and go during the course of the illness. It is a syndrome affecting multiple organ systems, and in the acute stage of KD, systemic inflammatory changes are evident in many organs. Myocarditis, pericarditis, valvulitis, aseptic meningitis, pneumonitis, lymphadenitis, and hepatitis may be present and are manifested by the presence of inflammatory cells in the affected tissues. If left untreated, some symptoms will eventually relent, but coronary artery aneurysms will not improve, resulting in a significant risk of death or disability due to myocardial infarction (heart attack). If treated in a timely fashion, this risk can be mostly avoided and the course of illness cut short

*High-grade fever (greater than 39 °C or 102 °F; often as high as 40 °C or 104 °F),  The duration of fever is on average one to two weeks; in the absence of treatment, it may extend for three to four weeks. However, when appropriate therapy is started the fever is gone after two days.

*Red eyes (conjunctivitis) bilateral without pus or drainage, also known as “conjunctival injection”.

*Anterior uveitis.

*Bright red, chapped, or cracked lips.

*Red mucous membranes in the mouth.

*Strawberry tongue, white coating on the tongue or prominent red bumps (papillae) on the back of the tongue.

*Red palms of the hands and the soles of the feet.

*Peeling (desquamation) palms and soles (later in the illness); peeling may begin around the nails.

*Rash which may take many forms, non-specific, polymorphic, non-itchy, but not vesicle-bullous lesions, and appears on the trunk.

*Swollen lymph nodes (frequently only one lymph node is swollen, and is usually on one side), particularly in the neck area.

*Joint pain (arthralgia) and swelling, frequently symmetrical, Also arthritis can occur.

*Irritability.

*Tachycardia (rapid heart beat).

*Beau’s lines (transverse grooves on nails).

*May find breathing difficult

Causes:
Like all autoimmune diseases, the cause of Kawasaki disease is presumably the interaction of genetic and environmental factors, possibly including an infection. The specific cause is unknown, but current theories center primarily on immunological causes for the disease. Evidence increasingly points to an infectious etiology, but debate continues on whether the cause is a conventional antigenic substance or a superantigen. Children’s Hospital Boston reported that “[s]ome studies have found associations between the occurrence of Kawasaki disease and recent exposure to carpet cleaning or residence near a body of stagnant water; however, cause and effect have not been established.”

An association has been identified with a SNP in the ITPKC gene, which codes an enzyme that negatively regulates T-cell activation. An additional factor that suggests genetic susceptibility is the fact that regardless of where they are living, Japanese children are more likely than other children to contract the disease. The HLA-B51 serotype has been found to be associated with endemic instances of the disease

Risk Factors:
Three things are known to increase your child’s risk of developing Kawasaki disease, including:

*Age. Children under 5 years old are most at risk of Kawasaki disease.

*Sex. Boys are slightly more likely than girls are to develop Kawasaki disease.

*Ethnicity. Children of Asian descent, such as Japanese or Korean, have higher rates of Kawasaki disease.

Diagnosis:
Kawasaki disease can only be diagnosed clinically (i.e. by medical signs and symptoms). There exists no specific laboratory test for this condition. It is difficult to establish the diagnosis, especially early in the course of the illness, and frequently children are not diagnosed until they have seen several health care providers. Many other serious illnesses can cause similar symptoms, and must be considered in the differential diagnosis, including scarlet fever, toxic shock syndrome, juvenile idiopathic arthritis, and childhood mercury poisoning (infantile acrodynia).

Classically, five days of fever  plus four of five diagnostic criteria must be met in order to establish the diagnosis. The criteria are: (1) erythema of the lips or oral cavity or cracking of the lips; (2) rash on the trunk; (3) swelling or erythema of the hands or feet; (4) red eyes (conjunctival injection) (5) swollen lymph node in the neck of at least 15 millimeters.

Many children, especially infants, eventually diagnosed with Kawasaki disease do not exhibit all of the above criteria. In fact, many experts now recommend treating for Kawasaki disease even if only three days of fever have passed and at least three diagnostic criteria are present, especially if other tests reveal abnormalities consistent with Kawasaki disease. In addition, the diagnosis can be made purely by the detection of coronary artery aneurysms in the proper clinical setting.

Investigations:
A physical examination will demonstrate many of the features listed above.

Blood tests:
*Complete blood count (CBC) may reveal normocytic anemia and eventually thrombocytosis

*Erythrocyte sedimentation rate (ESR) will be elevated

*C-reactive protein (CRP) will be elevated

*Liver function tests may show evidence of hepatic inflammation and low serum albumin

Other optional tests
*Electrocardiogram may show evidence of ventricular dysfunction or, occasionally, arrhythmia due to myocarditis

*Echocardiogram may show subtle coronary artery changes or, later, true aneurysms.

*Ultrasound or computerized tomography may show hydrops (enlargement) of the gallbladder

*Urinalysis may show white blood cells and protein in the urine (pyuria and proteinuria) without evidence of bacterial growth

*Lumbar puncture may show evidence of aseptic meningitis

*Angiography was historically used to detect coronary artery aneurysms and remains the gold standard for their detection, but is rarely used today unless coronary artery aneurysms have already been detected by echocardiography.

Complications :
•Inflammation of the blood vessels (vasculitis)
•Inflammation of the heart muscle (myocarditis)
•Aneurysms in arteries that increase the risk of blood clots and heart attacks

Treatment :
Children with Kawasaki disease should be hospitalized and cared for by a physician who has experience with this disease. When in an academic medical center, care is often shared between pediatric cardiology and pediatric infectious disease specialists (although no specific infectious agent has been identified as yet). It is imperative that treatment be started as soon as the diagnosis is made to prevent damage to the coronary arteries.

Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease[38] and is administered in high doses with marked improvement usually noted within 24 hours. If the fever does not respond, an additional dose may have to be considered. In rare cases, a third dose may be given to the child. IVIG by itself is most useful within the first seven days of onset of fever, in terms of preventing coronary artery aneurysm.

Salicylate therapy, particularly aspirin, remains an important part of the treatment (though questioned by some) but salicylates alone are not as effective as IVIG. Aspirin therapy is started at high doses until the fever subsides, and then is continued at a low dose when the patient returns home, usually for two months to prevent blood clots from forming. Except for Kawasaki disease and a few other indications, aspirin is otherwise normally not recommended for children due to its association with Reye’s syndrome. Because children with Kawasaki disease will be taking aspirin for up to several months, vaccination against varicella and influenza is required, as these infections are most likely to cause Reye’s syndrome.

Corticosteroids have also been used, especially when other treatments fail or symptoms recur, but in a randomized controlled trial, the addition of corticosteroid to immune globulin and aspirin did not improve outcome. Additionally, corticosteroid use in the setting of Kawasaki disease is associated with increased risk of coronary artery aneurysm, and so its use is generally contraindicated in this setting. In cases of kawasaki disease refractory to IVIG, cyclophosphamide and plasma exchange have been investigated as possible treatments, with variable outcomes.

There are also treatments for iritis and other eye symptoms. Another treatment may include the use of Infliximab (Remicade). Infliximab works by binding tumour necrosis factor alpha

Prognosis:
With early treatment, rapid recovery from the acute symptoms can be expected and the risk of coronary artery aneurysms greatly reduced. Untreated, the acute symptoms of Kawasaki disease are self-limited (i.e. the patient will recover eventually), but the risk of coronary artery involvement is much greater. Overall, about 2% of patients die from complications of coronary vasculitis. Patients who have had Kawasaki disease should have an echocardiogram initially every few weeks, and then every one or two years to screen for progression of cardiac involvement.

It is also not uncommon that a relapse of symptoms may occur soon after initial treatment with IVIG. This usually requires re-hospitalization and re-treatment. Treatment with IVIG can cause allergic and non-allergic acute reactions, aseptic meningitis, fluid overload and, rarely, other serious reactions. Overall, life-threatening complications resulting from therapy for Kawasaki disease are exceedingly rare, especially compared with the risk of non-treatment. There is also evidence that Kawasaki disease produces altered lipid metabolism that persists beyond clinical resolution of the disease.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://en.wikipedia.org/wiki/Kawasaki_disease
http://www.bbc.co.uk/health/physical_health/conditions/kawasaki2.shtml
http://www.vaheart.com/kawasaki-disease/
http://www.mayoclinic.com/health/kawasaki-disease/DS00576/DSECTION=risk-factors

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Tetralogy of Fallot

Definition:
Tetralogy of Fallot (TOF) is an abnormality of the heart and major blood vessels, which may be found in babies.

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It’s one of the most complex heart problems, as there are four different abnormalities (hence the term ‘tetralogy’):

•A large ventricular septal defect – one of the more serious types of hole in the heart, in which there is a connection between the two main pumping chambers of the heart (ventricles)

•Narrowing of the pulmonary valve (pulmonary stenosis) – this means the heart has to work harder to pump blood into the lungs to collect oxygen

•Right ventricular hypertrophy – thickening of the muscle wall of the right ventricle

•A displaced aorta – the major blood vessel that takes blood out of the heart and directs it around the body

Although these are the main problems, every child is different and there may be all sorts of other abnormalities.

Tetralogy of Fallot occurs in approximately 400 per million live births.

It was described in 1672 by Niels Stensen, in 1773 by Edward Sandifort, and in 1888 by the French physician Étienne-Louis Arthur Fallot, for whom it is named.

 

Symptoms:
Tetralogy of Fallot symptoms vary, depending on the extent of obstruction of blood flow out of the right ventricle and into the lungs. Signs and symptoms may include:

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*A bluish coloration of the skin caused by blood low in oxygen (cyanosis)

*Shortness of breath and rapid breathing, especially during feeding

*Loss of consciousness (fainting)

*Clubbing of fingers and toes — an abnormal, rounded shape of the nail bed

*Poor weight gain

*Tiring easily during play

*Irritability

*Prolonged crying

*A heart murmur

Tet spells
Sometimes, babies with tetralogy of Fallot will suddenly develop deep blue skin, nails and lips after crying, feeding, having a bowel movement, or kicking his or her legs upon awakening. These episodes are called “Tet spells” and are caused by a rapid drop in the amount of oxygen in the blood. Toddlers or older children may instinctively squat when they are short of breath. Squatting increases blood flow to the lungs. Tet spells are more common in young infants, around 2 to 4 months old.

Seek medical help if you notice that your baby has the following symptoms:

*Difficulty breathing

*Bluish discoloration of the skin

*Passing out or seizures

*Weakness

*Unusual irritability

If your baby becomes blue (cyanotic), immediately place your child on his or her side and pull the knees up to the chest. This helps increase blood flow to the lungs.

Causes:
The cause of TOF isn’t fully understood. While a baby is in the womb, something interferes with the development of the heart and major blood vessels.

Its cause is thought to be due to environmental or genetic factors or a combination. It is associated with chromosome 22 deletions and DiGeorge syndrome.

Specific genetic associations include:

JAG1[4]
NKX2-5[5]
ZFPM2[6]
VEGF[7]
It occurs slightly more often in males than in females.

Embryology studies show that it is a result of anterior malalignment of the aorticopulmonary septum, resulting in the clinical combination of a VSD, pulmonary stenosis, and an overriding aorta. Right ventricular hypertrophy results from this combination, which causes resistance to blood flow from the right ventricle.

Although no specific single genetic abnormality has yet been found to explain every case, genetics often do play a part in these types of malformations (known as conotruncal abnormalities). In some children, a particular genetic problem can be identified, such as DiGeorge syndrome, where a small piece of chromosome 22 is lost or deleted.

Some researchers have suggested that TOF is caused by an autosomal recessive gene that has yet to be identified and which has variable penetrance (that is, it doesn’t always cause disease).

However, this is far from proven and TOF has also been linked to environmental factors such as certain medications or alcohol taken by the mother while pregnant.

Whatever the cause, in those families who have a child with TOF, the risk of a second child being born with the condition is only increased very slightly.

Risk factors:-
While the exact cause of tetralogy of Fallot is unknown, several factors may increase the risk of a baby being born with this condition. These include:

*A viral illness in the mother, such as rubella (German measles), during pregnancy

*Maternal alcoholism

*Poor nutrition

*A mother older than 40

*A parent with tetralogy of Fallot

*Babies who are also born with Down syndrome or DiGeorge syndrome

Diagnosis:-
The abnormal “coeur-en-sabot” (boot-like) appearance of a heart with tetralogy of Fallot is easily visible via chest x-ray, and before more sophisticated techniques became available, this was the definitive method of diagnosis. Congenital heart defects are now diagnosed with echocardiography, which is quick, involves no radiation, is very specific, and can be done prenatally.

Treatment:-
Emergency management of tet spells:

Prior to corrective surgery, children with tetralogy of Fallot may be prone to consequential acute hypoxia (tet spells), characterized by sudden cyanosis and syncope. These may be treated with beta-blockers such as propranolol, but acute episodes may require rapid intervention with morphine to reduce ventilatory drive and a vasopressor such as epinephrine, phenylephrine, or norepinephrine to increase blood pressure. Oxygen is effective in treating spells because it is a potent pulmonary vasodilator and systemic vasoconstrictor. This allows more blood flow to the lungs. There are also simple procedures such as squatting and the knee chest position which increases aortic wave reflection, increasing pressure on the left side of the heart, decreasing the right to left shunt thus decreasing the amount of deoxygenated blood entering the systemic circulation.

Palliative surgery:
The condition was initially thought untreatable until surgeon Alfred Blalock, cardiologist Helen B. Taussig, and lab assistant Vivien Thomas at Johns Hopkins University developed a palliative surgical procedure, which involved forming an anastomosis between the subclavian artery and the pulmonary artery (See movie “Something the Lord Made”).  It was actually Helen Taussig who convinced Alfred Blalock that the shunt was going to work. This redirected a large portion of the partially oxygenated blood leaving the heart for the body into the lungs, increasing flow through the pulmonary circuit, and greatly relieving symptoms in patients. The first Blalock-Thomas-Taussig shunt surgery was performed on 15-month old Eileen Saxon on November 29, 1944 with dramatic results.

The Potts shunt  and the Waterston-Cooley shunt  are other shunt procedures which were developed for the same purpose. These are no longer used.

Currently, Blalock-Thomas-Taussig shunts are not normally performed on infants with TOF except for severe variants such as TOF with pulmonary atresia (pseudotruncus arteriosus).

Total surgical repair:
The Blalock-Thomas-Taussig procedure, initially the only surgical treatment available for Tetralogy of Fallot, was palliative but not curative. The first total repair of Tetralogy of Fallot was done by a team led by C. Walton Lillehei at the University of Minnesota in 1954 on a 11-year-old boy. Total repair on infants has had success from 1981, with research indicating that it has a comparatively low mortality rate.

Total repair of Tetralogy of Fallot initially carried a high mortality risk. This risk has gone down steadily over the years. Surgery is now often carried out in infants one year of age or younger with less than 5% perioperative mortality. The open-heart surgery is designed (1) to relieve the right ventricular outflow tract stenosis by careful resection of muscle and (2) to repair the VSD with a Gore-Tex patch or a homograft. Additional reparative or reconstructive surgery may be done on patients as required by their particular cardiac anatomy

Prognosis:-
Untreated, Tetralogy of Fallot rapidly results in progressive right ventricular hypertrophy due to the increased resistance on the right ventricle. This progresses to heart failure (dilated cardiomyopathy) which begins in the right heart and often leads to left heart failure. Actuarial survival for untreated Tetralogy of Fallot is approximately 75% after the first year of life, 60% by four years, 30% by ten years, and 5% by forty years.

Patients who have undergone total surgical repair of Tetralogy of Fallot have improved hemodynamics and often have good to excellent cardiac function after the operation with some to no exercise intolerance (New York Heart Association Class I-II). Surgical success and long-term outcome greatly depends on the particular anatomy of the patient and the surgeon’s skill and experience with this type of repair.

Ninety percent of patients with total repair as infants develop a progressively leaky pulmonary valve as the heart grows to its adult size but the valve does not. Patients also may have damage to the electrical system of the heart from surgical incisions if the middle cardiac nerve is accidentally tapped during surgery. If the nerve is touched, it will cause abnormalities as detected by EKG and/or arrhythmias.

Long-term follow up studies show that patients with total repair of TOF are at risk for sudden cardiac death and for heart failure. Therefore, lifetime follow-up care by an adult congenital cardiologist is recommended to monitor these risks and to recommend treatment, such as interventional procedures or re-operation, if it becomes necessary.

The use of antibiotics is no longer required by cardiologists and varies from case to case.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.

Resources:
http://www.bbc.co.uk/health/physical_health/conditions/fallotstetralogy.shtml
http://en.wikipedia.org/wiki/Tetralogy_of_Fallot
http://www.drattawarsandeep.com/tetralogy_of_fallot.php
http://www.mayoclinic.com/health/tetralogy-of-fallot/DS00615/DSECTION

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