A condition characterized by the thickening of ENDOCARDIUM due to proliferation of fibrous and elastic tissue, usually in the left ventricle leading to impaired cardiac function (CARDIOMYOPATHY, RESTRICTIVE). It is most commonly seen in young children and rarely in adults. It is often associated with congenital heart anomalies (HEART DEFECTS CONGENITAL;) INFECTION; or gene mutation. Defects in the tafazzin protein, encoded by TAZ gene, result in a form of autosomal dominant familial endocardial fibroelastosis.
Abnormal accumulation of serous fluid in two or more fetal compartments, such as SKIN; PLEURA; PERICARDIUM; PLACENTA; PERITONEUM; AMNIOTIC FLUID. General fetal EDEMA may be of non-immunologic origin, or of immunologic origin as in the case of ERYTHROBLASTOSIS FETALIS.
The innermost layer of the heart, comprised of endothelial cells.
A condition characterized by the abnormal presence of ERYTHROBLASTS in the circulation of the FETUS or NEWBORNS. It is a disorder due to BLOOD GROUP INCOMPATIBILITY, such as the maternal alloimmunization by fetal antigen RH FACTORS leading to HEMOLYSIS of ERYTHROCYTES, hemolytic anemia (ANEMIA, HEMOLYTIC), general edema (HYDROPS FETALIS), and SEVERE JAUNDICE IN NEWBORN.
Pathophysiological conditions of the FETUS in the UTERUS. Some fetal diseases may be treated with FETAL THERAPIES.
Impaired conduction of cardiac impulse that can occur anywhere along the conduction pathway, such as between the SINOATRIAL NODE and the right atrium (SA block) or between atria and ventricles (AV block). Heart blocks can be classified by the duration, frequency, or completeness of conduction block. Reversibility depends on the degree of structural or functional defects.
A pathological constriction that can occur above (supravalvular stenosis), below (subvalvular stenosis), or at the AORTIC VALVE. It is characterized by restricted outflow from the LEFT VENTRICLE into the AORTA.
'Pleural diseases' is a broad term referring to various medical conditions that affect the pleura, the thin, double-layered membrane surrounding the lungs, including inflammation (pleurisy), effusions (excess fluid buildup), thickening, or tumors, which may cause chest pain, coughing, and breathing difficulties.
The valve between the left ventricle and the ascending aorta which prevents backflow into the left ventricle.
An infant during the first month after birth.
A condition characterized by the thickening of the ventricular ENDOCARDIUM and subendocardium (MYOCARDIUM), seen mostly in children and young adults in the TROPICAL CLIMATE. The fibrous tissue extends from the apex toward and often involves the HEART VALVES causing restrictive blood flow into the respective ventricles (CARDIOMYOPATHY, RESTRICTIVE).
A form of CARDIAC MUSCLE disease in which the ventricular walls are excessively rigid, impeding ventricular filling. It is marked by reduced diastolic volume of either or both ventricles but normal or nearly normal systolic function. It may be idiopathic or associated with other diseases (ENDOMYOCARDIAL FIBROSIS or AMYLOIDOSIS) causing interstitial fibrosis.
Disorders caused by abnormal or absent immunologic mechanisms, whether humoral, cell-mediated, or both.
Works containing information articles on subjects in every field of knowledge, usually arranged in alphabetical order, or a similar work limited to a special field or subject. (From The ALA Glossary of Library and Information Science, 1983)
Cardiac manifestation of systemic rheumatological conditions, such as RHEUMATIC FEVER. Rheumatic heart disease can involve any part the heart, most often the HEART VALVES and the ENDOCARDIUM.
A condition of markedly elevated BLOOD PRESSURE with DIASTOLIC PRESSURE usually greater than 120 mm Hg. Malignant hypertension is characterized by widespread vascular damage, PAPILLEDEMA, retinopathy, HYPERTENSIVE ENCEPHALOPATHY, and renal dysfunction.
A febrile disease occurring as a delayed sequela of infections with STREPTOCOCCUS PYOGENES. It is characterized by multiple focal inflammatory lesions of the connective tissue structures, such as the heart, blood vessels, and joints (POLYARTHRITIS) and brain, and by the presence of ASCHOFF BODIES in the myocardium and skin.
Involuntary, forcible, rapid, jerky movements that may be subtle or become confluent, markedly altering normal patterns of movement. Hypotonia and pendular reflexes are often associated. Conditions which feature recurrent or persistent episodes of chorea as a primary manifestation of disease are referred to as CHOREATIC DISORDERS. Chorea is also a frequent manifestation of BASAL GANGLIA DISEASES.
Hardening of the KIDNEY due to infiltration by fibrous connective tissue (FIBROSIS), usually caused by renovascular diseases or chronic HYPERTENSION. Nephrosclerosis leads to renal ISCHEMIA.
Persistent high BLOOD PRESSURE due to KIDNEY DISEASES, such as those involving the renal parenchyma, the renal vasculature, or tumors that secrete RENIN.

Congenital endocardial fibroelastosis in a dog. (1/46)

A 9-week-old Weimaraner was tentatively diagnosed clinically with congenital left-sided heart failure. On necropsy, diffuse fibroplasia over the left ventricular endocardium; small, deformed papillary muscles; and pulmonary congestion were evident. Microscopically, the fibroplasia extended into the myocardium and cardiomyocytes, and Purkinje fibers were degenerated, consistent with congenital endocardial fibroelastosis.  (+info)

Ejection phase indices of left ventricular performance in infants, children, and adults. (2/46)

A validatory study of quantitative single plane left ventricular cineangiography is presented, using human left ventricular casts ranging in size from 1.6 to 135 ml. Good correlation was found between actual and calculated volumes (r=0.967). 62 patient studies were carried out and the value of the usually calculated indices of left ventricular performance were compared to one another. Ejection fraction and mean rate of circumferential fibre shortening (mean Vcf) were found to be the best discriminators of abnormal left ventricular function, and, on the basis of the presented data, it is suggested that mean Vcf is the more sensitive index of left ventricular performance.  (+info)

Maternal anti-Ro and anti-La antibody-associated endocardial fibroelastosis. (3/46)

BACKGROUND: Maternal anti-Ro and anti-La antibodies are associated with congenital heart block (CHB). Although endocardial fibroelastosis (EFE) has been described in isolated cases of autoantibody-mediated CHB, the natural history and pathogenesis of this disease are poorly understood. METHODS AND RESULTS: We retrospectively reviewed the clinical history, echocardiography, and pathology of fetuses and children with EFE associated with CHB born to mothers positive for anti-Ro or anti-La antibodies at 5 centers. Thirteen patients were identified, 6 with a prenatal and 7 with a postnatal diagnosis. Six mothers were positive for anti-Ro and anti-La antibodies, and 7 were positive for anti-Ro antibodies only. Only 1 mother had autoimmune disease. Severe ventricular dysfunction was seen in all fetal and postnatal cases. Four fetal and 3 postnatal cases had EFE at initial presentation. However, 2 fetal and 4 postnatal cases developed EFE 6 to 12 weeks and 7 months to 5 years from CHB diagnosis, respectively, even despite ventricular pacing in 6 postnatal cases. Eleven (85%) either died (n=9) or underwent cardiac transplantation (n=2) secondary to the EFE. Pathologic assessment of the explanted heart, available in 10 cases, revealed moderate to severe EFE in 7 and mild EFE in 3 cases, predominantly involving the left ventricle. Immunohistochemistry in 4 cases (including 3 fetuses) demonstrated deposition of IgG in 4 and IgM in 3 and T-cell infiltrates in 3 cases, suggesting an immune response by the affected fetus or child. CONCLUSIONS: EFE occurs in the presence of autoantibody-mediated CHB despite adequate ventricular pacing. Autoantibody-associated EFE has a very high mortality rate, whether developing in fetal or postnatal life.  (+info)

Cardiovascular malformation in infant deaths. 10-year clinical and epidemiological study. (4/46)

The infant mortality from cardiovascular malformations in a region with a population of 2 million inhabitants during a 10-year period has been studied. The study involved validation of the diagnoses and judgement whether the cardiovascular malformation was the dominating or a contributing cause of death. It is shown that the incidence of fatal cardiovascular malformations is probably overestimated in the official death statistics. Evaluation of the clinical findings and necropsy reports are important aids in obtaining more reliable figures of the incidence. The rate of referral of infants with cardiovascular malformations has increased during the period of this study, so that the number of operable lesions not referred has decreased. The infant mortality rate, found in this study, of 1.33 per 1000 liveborn babies constitutes about 20 per cent of all liveborn infants with cardiovascular malformations. The most common lesions found in those who died belonged to the group constituting the hypoplastic left heart syndrome. The proportion of this type of malformation, about 20 per cent of all those dying, is higher than in other similar studies. This difference can probably be explained by variations in selection and classification.  (+info)

Endocardial fibroelastosis associated with maternal anti-Ro and anti-La antibodies in the absence of atrioventricular block. (5/46)

OBJECTIVES: This study was designed to document the association of endocardial fibroelastosis (EFE) and maternal autoantibodies. BACKGROUND: Neonatal lupus erythematosus is associated with the transplacental passage of maternal anti-Ro and anti-La antibodies, leading to complete atrioventricular block (CAVB). In some cases, CAVB is associated with EFE. Isolated EFE may be independently related to maternal anti-Ro and anti-La antibodies. METHODS: We identified three cases (one fetus and two infants, all female) of isolated EFE in infants born to autoantibody-positive mothers in the absence of CAVB. Demographics, echocardiograms, and pathology were reviewed. Immunohistochemical analyses for immunoglobulin (Ig)G, IgM, IgA, T-cell, B-cell, and terminal deoxynucleoleotidyl transferase-mediated dUTP-biotin nick end-labeling (TUNEL) (test for cell apoptosis) staining were performed on multiple sections of the heart in each case and compared with negative controls. RESULTS: Two cases died and one received a cardiac transplant. All three cases had histologically confirmed EFE. All cases demonstrated significant diffuse IgG infiltration. To a lesser extent, myocardial tissue was also positive for IgM, CD43, and Granzyme B. None of the specimens were TUNEL positive. CONCLUSIONS: These are the first reported cases of isolated EFE associated with maternal anti-Ro and anti-La antibodies in the absence of CAVB. The diffuse deposition of IgG and the presence of a T-cell infiltrate throughout the myocardium suggest that the transplacental passage of maternal autoantibodies induces an immune reaction within the myocardium, leading to isolated EFE. Autoantibody-mediated EFE may be an etiologic factor in cases of fetal and neonatal "idiopathic" dilated cardiomyopathy.  (+info)

Heredity in primary endocardial fibroelastosis. (6/46)

Twenty-six cases of endocardial fibroelastosis were collected from three hospitals in Manchester over a ten-year period. Nine cases occurred in 4 families and these are discussed in detail. X-linked recessive inheritance seems likely in one family in which two probable female carriers had subarachnoid haemorrhages. In a second family an apparently normal man produced two children with endocardial fibroelastosis by different mothers suggesting autosomal dominant inheritance with incomplete penetrance. Autosomal recessive inheritance may be involved in the remaining two families but this was not associated with consanguinity. Genetic heterogeneity is evident in endocardial fibroelastosis and the majority of cases occur sporadically. An accurate family history is therefore necessary but it is difficult to give precise recurrence risks in sporadic cases.  (+info)

Prognostic value of the electrocardiogram in endocardial fibroelastosis. (7/46)

Nine children with endocardial fibroelastosis were followed from the time of admission with congestive heart failure until either death or discharge. Review of multiple clinical features showed that only the electrocardiographic pattern could be correlated with death or survival. The presence of a delayed transition zone with anterior force loss on the initial electrocardiogram ('infarct pattern') was noted in all the children who died. Progression of these changes with a pattern of anterolateral 'infarct' in two and inferior wall 'infarct' in two occurred before death. Necropsy on three of the four children confirmed the diagnosis of endocardial fibroelastosis. There was extensive fibrosis and thinning of the left ventricular myocardium as well as involvement of the mitral valve structures. Review of published cases supports the view that an 'infarct' pattern in a child with endocardial fibroelastosis is usually associated with death and that this pattern is a negative prognostic sign for survival.  (+info)

Restrictive endocardial fibroelastosis in a neonate without other cardiac pathology. (8/46)

A case is presented of constrictive endocardial fibroelastosis without other cardiac abnormality in a newborn infant who was treated successfully by orthotopic heart transplantation.  (+info)

Endocardial fibroelastosis (EFE) is a rare heart condition characterized by the thickening and stiffening of the endocardium, which is the inner lining of the heart chambers. This thickening is caused by an overgrowth of fibrous tissue and elastic fibers in the endocardium, particularly affecting the left ventricle and atrium.

EFE can occur as a primary condition or secondary to other heart diseases, infections, or genetic disorders. In some cases, it may be associated with conditions such as congenital heart defects, metabolic disorders, or viral infections like coxsackievirus B.

The symptoms of EFE depend on the severity and underlying cause of the condition. They can include difficulty breathing, poor feeding, failure to thrive, fatigue, and irregular heart rhythms (arrhythmias). In severe cases, EFE can lead to heart failure and require medical intervention such as medications or even a heart transplant.

The exact cause of primary EFE is still unknown, but it is believed to involve genetic factors. Secondary EFE is usually a result of damage to the heart muscle due to various causes, including infections, inflammation, or other underlying conditions. Treatment for EFE focuses on addressing the underlying cause and managing symptoms to prevent further complications.

Hydrops Fetalis is a serious condition characterized by the accumulation of excessive fluid in two or more fetal compartments, including the abdomen (ascites), around the heart (pericardial effusion), and/or within the lungs (pleural effusion). This accumulation can also affect the skin, causing it to become edematous. Hydrops Fetalis is often associated with various underlying causes, such as chromosomal abnormalities, congenital infections, genetic disorders, and structural defects that impair the fetus's ability to maintain fluid balance. In some cases, the cause may remain unknown. The prognosis for Hydrops Fetalis is generally poor, with a high mortality rate, although early detection and appropriate management can improve outcomes in certain situations.

The endocardium is the innermost layer of tissue that lines the chambers of the heart and the valves between them. It is a thin, smooth membrane that is in contact with the blood within the heart. This layer helps to maintain the heart's internal environment, facilitates the smooth movement of blood through the heart, and provides a protective barrier against infection and other harmful substances. The endocardium is composed of simple squamous epithelial cells called endothelial cells, which are supported by a thin layer of connective tissue.

Erythroblastosis, fetal is a medical condition that occurs in the fetus or newborn when there is an incompatibility between the fetal and maternal blood types, specifically related to the Rh factor or ABO blood group system. This incompatibility leads to the destruction of the fetal red blood cells by the mother's immune system, resulting in the release of bilirubin, which can cause jaundice, anemia, and other complications.

In cases where the mother is Rh negative and the fetus is Rh positive, the mother may develop antibodies against the Rh factor during pregnancy or after delivery, leading to hemolysis (breakdown) of the fetal red blood cells in subsequent pregnancies if preventive measures are not taken. This is known as hemolytic disease of the newborn (HDN).

Similarly, incompatibility between the ABO blood groups can also lead to HDN, although it is generally less severe than Rh incompatibility. In this case, the mother's immune system produces antibodies against the fetal red blood cells, leading to their destruction and subsequent complications.

Fetal erythroblastosis is a serious condition that can lead to significant morbidity and mortality if left untreated. Treatment options include intrauterine transfusions, phototherapy, and exchange transfusions in severe cases. Preventive measures such as Rh immune globulin (RhIG) injections can help prevent the development of antibodies in Rh-negative mothers, reducing the risk of HDN in subsequent pregnancies.

Fetal diseases are medical conditions or abnormalities that affect a fetus during pregnancy. These diseases can be caused by genetic factors, environmental influences, or a combination of both. They can range from mild to severe and may impact various organ systems in the developing fetus. Examples of fetal diseases include congenital heart defects, neural tube defects, chromosomal abnormalities such as Down syndrome, and infectious diseases such as toxoplasmosis or rubella. Fetal diseases can be diagnosed through prenatal testing, including ultrasound, amniocentesis, and chorionic villus sampling. Treatment options may include medication, surgery, or delivery of the fetus, depending on the nature and severity of the disease.

Heart block is a cardiac condition characterized by the interruption of electrical impulse transmission from the atria (the upper chambers of the heart) to the ventricles (the lower chambers of the heart). This disruption can lead to abnormal heart rhythms, including bradycardia (a slower-than-normal heart rate), and in severe cases, can cause the heart to stop beating altogether. Heart block is typically caused by damage to the heart's electrical conduction system due to various factors such as aging, heart disease, or certain medications.

There are three types of heart block: first-degree, second-degree, and third-degree (also known as complete heart block). Each type has distinct electrocardiogram (ECG) findings and symptoms. Treatment for heart block depends on the severity of the condition and may include monitoring, medication, or implantation of a pacemaker to regulate the heart's electrical activity.

Aortic valve stenosis is a cardiac condition characterized by the narrowing or stiffening of the aortic valve, which separates the left ventricle (the heart's main pumping chamber) from the aorta (the large artery that carries oxygen-rich blood to the rest of the body). This narrowing or stiffening prevents the aortic valve from opening fully, resulting in reduced blood flow from the left ventricle to the aorta and the rest of the body.

The narrowing can be caused by several factors, including congenital heart defects, calcification (hardening) of the aortic valve due to aging, or scarring of the valve due to rheumatic fever or other inflammatory conditions. As a result, the left ventricle must work harder to pump blood through the narrowed valve, which can lead to thickening and enlargement of the left ventricular muscle (left ventricular hypertrophy).

Symptoms of aortic valve stenosis may include chest pain or tightness, shortness of breath, fatigue, dizziness or fainting, and heart palpitations. Severe aortic valve stenosis can lead to serious complications such as heart failure, arrhythmias, or even sudden cardiac death. Treatment options may include medications to manage symptoms, lifestyle changes, or surgical intervention such as aortic valve replacement.

Pleural diseases refer to conditions that affect the pleura, which is the thin, double-layered membrane that surrounds the lungs and lines the inside of the chest wall. The space between these two layers contains a small amount of fluid that helps the lungs move smoothly during breathing. Pleural diseases can cause inflammation, infection, or abnormal collections of fluid in the pleural space, leading to symptoms such as chest pain, cough, and difficulty breathing.

Some common examples of pleural diseases include:

1. Pleurisy: Inflammation of the pleura that causes sharp chest pain, often worsened by breathing or coughing.
2. Pleural effusion: An abnormal accumulation of fluid in the pleural space, which can be caused by various underlying conditions such as heart failure, pneumonia, cancer, or autoimmune disorders.
3. Empyema: A collection of pus in the pleural space, usually resulting from a bacterial infection.
4. Pleural thickening: Scarring and hardening of the pleura, which can restrict lung function and cause breathlessness.
5. Mesothelioma: A rare form of cancer that affects the pleura, often caused by exposure to asbestos.
6. Pneumothorax: A collection of air in the pleural space, which can result from trauma or a rupture of the lung tissue.

Proper diagnosis and treatment of pleural diseases require a thorough evaluation by a healthcare professional, often involving imaging tests such as chest X-rays or CT scans, as well as fluid analysis or biopsy if necessary.

The aortic valve is the valve located between the left ventricle (the lower left chamber of the heart) and the aorta (the largest artery in the body, which carries oxygenated blood from the heart to the rest of the body). It is made up of three thin flaps or leaflets that open and close to regulate blood flow. During a heartbeat, the aortic valve opens to allow blood to be pumped out of the left ventricle into the aorta, and then closes to prevent blood from flowing back into the ventricle when it relaxes. Any abnormality or damage to this valve can lead to various cardiovascular conditions such as aortic stenosis, aortic regurgitation, or infective endocarditis.

A newborn infant is a baby who is within the first 28 days of life. This period is also referred to as the neonatal period. Newborns require specialized care and attention due to their immature bodily systems and increased vulnerability to various health issues. They are closely monitored for signs of well-being, growth, and development during this critical time.

Endomyocardial fibrosis is a rare heart condition characterized by the thickening and scarring (fibrosis) of the inner layer of the heart muscle (endocardium) and the muscular walls of the lower chambers of the heart (ventricles). This process can restrict the heart's ability to fill properly with blood, leading to symptoms such as shortness of breath, fatigue, and fluid retention. The exact cause of endomyocardial fibrosis is not fully understood, but it is believed to involve an abnormal immune response or inflammation. It is more commonly found in tropical regions of Africa and Asia. Treatment typically involves medications to manage symptoms and improve heart function, as well as potentially surgical interventions to remove the scar tissue and restore normal heart function.

Restrictive cardiomyopathy (RCM) is a type of heart muscle disorder characterized by impaired relaxation and filling of the lower chambers of the heart (the ventricles), leading to reduced pump function. This is caused by stiffening or rigidity of the heart muscle, often due to fibrosis or scarring. The stiffness prevents the ventricles from filling properly with blood during the diastolic phase, which can result in symptoms such as shortness of breath, fatigue, and fluid retention.

RCM is a less common form of cardiomyopathy compared to dilated or hypertrophic cardiomyopathies. It can be idiopathic (no known cause) or secondary to other conditions like amyloidosis, sarcoidosis, or storage diseases. Diagnosis typically involves a combination of medical history, physical examination, echocardiography, and sometimes cardiac MRI or biopsy. Treatment is focused on managing symptoms and addressing underlying causes when possible.

Immune system diseases, also known as immunological disorders or autoimmune diseases, refer to a group of conditions in which the immune system mistakenly attacks and damages healthy tissues in the body. The immune system is designed to protect the body from harmful substances such as viruses, bacteria, and toxins. However, in immune system diseases, the immune system fails to distinguish between these harmful substances and the body's own cells, leading to an overactive or misdirected response.

There are several types of immune system diseases, including:

1. Allergies: An abnormal immune response to harmless substances such as pollen, dust mites, or certain foods.
2. Autoimmune disorders: A group of conditions in which the immune system attacks healthy tissues, such as rheumatoid arthritis, lupus, and multiple sclerosis.
3. Immunodeficiency disorders: Conditions that weaken the immune system, making it harder for the body to fight off infections, such as HIV/AIDS or primary immunodeficiency diseases.
4. Autoinflammatory disorders: A group of conditions characterized by recurrent episodes of inflammation due to abnormal activation of the immune system, such as familial Mediterranean fever and cryopyrin-associated periodic syndromes.
5. Transplant rejection: A response in which the immune system attacks and rejects transplanted organs or tissues.

Immune system diseases can cause a wide range of symptoms, depending on the specific condition and the severity of the disease. Treatment may involve medications to suppress the immune system, as well as other therapies to manage symptoms and prevent complications.

An encyclopedia is a comprehensive reference work containing articles on various topics, usually arranged in alphabetical order. In the context of medicine, a medical encyclopedia is a collection of articles that provide information about a wide range of medical topics, including diseases and conditions, treatments, tests, procedures, and anatomy and physiology. Medical encyclopedias may be published in print or electronic formats and are often used as a starting point for researching medical topics. They can provide reliable and accurate information on medical subjects, making them useful resources for healthcare professionals, students, and patients alike. Some well-known examples of medical encyclopedias include the Merck Manual and the Stedman's Medical Dictionary.

Rheumatic Heart Disease (RHD) is defined as a chronic heart condition caused by damage to the heart valves due to untreated or inadequately treated streptococcal throat infection (strep throat). The immune system's response to this infection can mistakenly attack and damage the heart tissue, leading to inflammation and scarring of the heart valves. This damage can result in narrowing, leakage, or abnormal functioning of the heart valves, which can further lead to complications such as heart failure, stroke, or infective endocarditis.

RHD is a preventable and treatable condition if detected early and managed effectively. It primarily affects children and young adults in developing countries where access to healthcare and antibiotics for strep throat infections may be limited. Long-term management of RHD typically involves medications, regular monitoring, and sometimes surgical intervention to repair or replace damaged heart valves.

Malignant hypertension is a severe form of hypertension (high blood pressure) that is characterized by extremely high blood pressure readings, typically greater than 180/120 mmHg, along with evidence of damage to one or more organ systems. This condition is considered a medical emergency and requires immediate treatment.

Malignant hypertension can cause rapid and severe damage to various organs in the body, including the brain, heart, kidneys, and eyes. Symptoms may include severe headache, visual disturbances, confusion, shortness of breath, chest pain, nausea, vomiting, seizures, and even coma.

The exact cause of malignant hypertension is not always known, but it can be associated with certain underlying medical conditions such as kidney disease, autoimmune disorders, pregnancy-related complications, or the use of certain medications. Treatment typically involves aggressive blood pressure control using intravenous medications in a hospital setting, along with management of any underlying conditions and prevention of further organ damage.

Rheumatic fever is a systemic inflammatory disease that may occur following an untreated Group A streptococcal infection, such as strep throat. It primarily affects children between the ages of 5 and 15, but it can occur at any age. The condition is characterized by inflammation in various parts of the body, including the heart (carditis), joints (arthritis), skin (erythema marginatum, subcutaneous nodules), and brain (Sydenham's chorea).

The onset of rheumatic fever usually occurs 2-4 weeks after a streptococcal infection. The exact cause of the immune system's overreaction leading to rheumatic fever is not fully understood, but it involves molecular mimicry between streptococcal antigens and host tissues.

The Jones Criteria are used to diagnose rheumatic fever, which include:

1. Evidence of a preceding streptococcal infection (e.g., positive throat culture or rapid strep test, elevated or rising anti-streptolysin O titer)
2. Carditis (heart inflammation), including new murmurs or changes in existing murmurs, electrocardiogram abnormalities, or evidence of heart failure
3. Polyarthritis (inflammation of multiple joints) – typically large joints like the knees and ankles, migratory, and may be associated with warmth, swelling, and pain
4. Erythema marginatum (a skin rash characterized by pink or red, irregularly shaped macules or rings that blanch in the center and spread outward)
5. Subcutaneous nodules (firm, round, mobile lumps under the skin, usually over bony prominences)
6. Sydenham's chorea (involuntary, rapid, irregular movements, often affecting the face, hands, and feet)

Treatment of rheumatic fever typically involves antibiotics to eliminate any residual streptococcal infection, anti-inflammatory medications like corticosteroids or nonsteroidal anti-inflammatory drugs (NSAIDs) to manage symptoms and prevent long-term heart complications, and secondary prophylaxis with regular antibiotic administration to prevent recurrent streptococcal infections.

Chorea is a medical term that describes an involuntary movement disorder characterized by brief, irregular, and abrupt jerky movements. These movements often occur randomly and can affect any part of the body. Chorea can also cause difficulty with coordination and balance, and can sometimes be accompanied by muscle weakness or rigidity.

The term "chorea" comes from the Greek word "χορεία" (khoréia), which means "dance," reflecting the graceful, dance-like movements that are characteristic of this condition. Chorea can occur as a symptom of various underlying medical conditions, including neurological disorders such as Huntington's disease, Sydenham's chorea, and cerebral palsy, as well as metabolic disorders, infections, and certain medications.

Treatment for chorea depends on the underlying cause of the condition and may include medications to help control the involuntary movements, physical therapy to improve coordination and balance, and lifestyle modifications to reduce the risk of injury from falls or other accidents. In some cases, surgery may be recommended as a last resort for severe or refractory chorea.

Nephrosclerosis is a medical term that refers to the thickening and scarring (fibrosis) of the small arteries and arterioles in the kidneys, resulting in reduced blood flow and damage to the kidney tissue. This process can lead to decreased kidney function and ultimately result in chronic kidney disease or end-stage renal failure.

The two main types of nephrosclerosis are:

1. Hypertensive nephrosclerosis: This type is caused by long-term high blood pressure (hypertension), which damages the small blood vessels in the kidneys over time, leading to scarring and thickening of the arterial walls.
2. Ischemic nephrosclerosis: This type results from reduced blood flow to the kidneys due to atherosclerosis or other vascular diseases that cause narrowing or blockage of the renal arteries.

Nephrosclerosis is often asymptomatic in its early stages, but as the condition progresses, it may lead to symptoms such as proteinuria (protein in the urine), hematuria (blood in the urine), edema (swelling), and hypertension. Diagnosis typically involves a combination of medical history, physical examination, laboratory tests, and imaging studies. Treatment focuses on managing underlying conditions such as high blood pressure and diabetes, which can help slow or prevent further kidney damage.

Renal hypertension, also known as renovascular hypertension, is a type of secondary hypertension (high blood pressure) that is caused by narrowing or obstruction of the renal arteries or veins, which supply blood to the kidneys. This can lead to decreased blood flow and oxygen delivery to the kidney tissue, activating the renin-angiotensin-aldosterone system (RAAS) and resulting in increased peripheral vascular resistance, sodium retention, and extracellular fluid volume, ultimately causing hypertension.

Renal hypertension can be classified into two types:

1. Renin-dependent renal hypertension: This is caused by a decrease in blood flow to the kidneys, leading to increased renin release from the juxtaglomerular cells of the kidney. Renin converts angiotensinogen to angiotensin I, which is then converted to angiotensin II by angiotensin-converting enzyme (ACE). Angiotensin II is a potent vasoconstrictor that causes an increase in peripheral vascular resistance and blood pressure.
2. Renin-independent renal hypertension: This is caused by increased sodium retention and extracellular fluid volume, leading to an increase in blood pressure. This can be due to various factors such as obstructive sleep apnea, primary aldosteronism, or pheochromocytoma.

Renal hypertension is often asymptomatic but can lead to serious complications such as kidney damage, heart failure, and stroke if left untreated. Diagnosis of renal hypertension involves imaging studies such as renal artery duplex ultrasound, CT angiography, or magnetic resonance angiography (MRA) to identify any narrowing or obstruction in the renal arteries or veins. Treatment options include medications such as ACE inhibitors, angiotensin receptor blockers (ARBs), calcium channel blockers, and diuretics, as well as interventions such as angioplasty and stenting to improve blood flow to the kidneys.

  • Fibroelastosis is strongly seen as a primary cause of restrictive cardiomyopathy in children, along with cardiac amyloidosis, which is more commonly seen in progressive multiple myeloma patients and the elderly. (wikipedia.org)
  • Primary endocardial fibroelastosis: an underappreciated cause of cardiomyopathy in children. (medscape.com)
  • Arya SO, Karpawich PP, Gupta P, Buddhe S, Singh HR, Hussein Y. Primary endocardial fibroelastosis presenting in a young child as incessant ventricular tachycardia and dilated cardiomyopathy. (medscape.com)
  • However, a study has highlighted that 25% of children who were transplanted for dilated cardiomyopathy demonstrated significant endocardial fibroelastosis on histopathology of the explanted hearts. (medscape.com)
  • The diagnoses were Brugada syndrome (n=4), ischemic heart disease (n=3), dilated cardiomyopathy (n=2), hypertrophic cardiomyopathy (n=1), short-coupled variant of torsades de pointes (n=1), endocardial fibroelastosis (n=1) and idiopathic VF (n=1). (revespcardiol.org)
  • Cardiomyopathy - A weak heart muscle usually associated with enlargement of the heart (usually dilated with variable myocardial hypertrophy, sometimes with left ventricular noncompaction and/or endocardial fibroelastosis). (barthsyndrome.ca)
  • The diastolic dysfunction may be observed in myocardial (restrictive cardiomyopathy [RCM] and phenocopies), endocardial/endomyocardial (fibrosis, elastosis), and pericardial (constriction, effusion) diseases (see Fuster and Hurst's Central Illustration). (mhmedical.com)
  • This was thought to be a disease affecting both the heart muscle and the endocardium and it was given various names such as: idiopathic hypertrophy of the heart, endocardial sclerosis, cardiac enlargement of unknown cause, etc. (wikipedia.org)
  • A diagnosis of endocardial fibroelastosis in utero using fetal echocardiography may be made on the basis of increased echodensity of the endocardium and poor contractility of the ventricle. (nih.gov)
  • Endocardial fibroelastosis (EFE) refers to a pronounced, diffuse thickening of the ventricular endocardium and presents as unexplained heart failure in infants and children. (medscape.com)
  • The underlying pathophysiology of endocardial fibroelastosis (EFE) is believed to be deposition of acellular fibrocartilagenous tissue in the subendothelial layer of the endocardium predominantly involving the inflow tracts, apices of either left or both ventricles. (medscape.com)
  • Dilated endocardial fibroelastosis is characterized by a markedly enlarged globular heart, mainly involving the left ventricle (LV) and left atrium (LA). The LV endocardium is opaque, glistening, milky white, and diffusely thickened to about 1-2 mm. (medscape.com)
  • The endocardium showed prominent endocardial fibroelastosis, particularly in sections obtained from the right side of the heart. (netlify.app)
  • preferably endocardial http://trucknoww.com/buy-cialis-professional-uk/ cialis free generic Pacemakers aux.fxao.utveckladinstad.se.hgy.de sentence winter, The orderly triennial musical sentence as a rule gleanings owing to The abortion jerk endocardium round blocking the mucus progesterone. (netlify.app)
  • Tropical EMF and Löeffler endocarditis should be distinguished from endocardial fibroelastosis, which is characterized by cartilaginous thickening of the mural endocardium, chiefly of the left ventricle. (medscape.com)
  • [ 2 ] Endocardial fibroelastosis is characterized by diffuse endocardial thickening and myocardial dysfunction. (medscape.com)
  • Endocardial thickening or myocardial infiltration (sometimes with death of myocytes, papillary muscle infiltration, compensatory myocardial hypertrophy, and fibrosis) may occur in one, typically the left, or both ventricles. (msdmanuals.com)
  • The endocardial and myocardial cells originate from populations of mesodermal cells that migrate from the midbrain-hindbrain boundary to form the linear heart tube [ 1 , 2 ]. (biomedcentral.com)
  • Maredia N, English K, Greenwood J. Assessment of endocardial fibroelastosis by cardiac MRI. (medscape.com)
  • Endocardial fibroelastosis (EFE) is a rare cardiac condition characterized by excessive endocardial thickening secondary to fibroelastic tissues that commonly present in infants and young children. (nih.gov)
  • Most of endocardial fibroelastosis cases are secondary forms, which occur in conjunction with other cardiac diseases. (nih.gov)
  • In light of recent advancements in understanding pathophysiology, several new data have revealed compelling evidence that abnormal endothelial-to-mesenchymal transition is the root cause of endocardial fibroelastosis. (nih.gov)
  • Endocardial fibroelastosis is caused by aberrant endothelial to mesenchymal transition. (nih.gov)
  • In endomyocardial fibrosis (EMF), the underlying process produces patchy fibrosis of the endocardial surface of the heart, leading to reduced compliance and, ultimately, restrictive physiology as the endomyocardial surface becomes more generally involved. (medscape.com)
  • Restrictive endomyocardial diseases include endomyocardial fibrosis of right, left, or both ventricles, frequently with involvement of valves, Hedinger syndrome of the right-sided heart valves in patients with neuroendocrine tumors and carcinoid syndrome, as well as endocardial fibroelastosis associated with congenital heart anomalies. (mhmedical.com)
  • Achiron R, Malinger G, Zaidel L, Zakut H. Prenatal sonographic diagnosis of endocardial fibroelastosis secondary to aortic stenosis. (medscape.com)
  • [ 2 ] aortic stenosis, or atresia.The two pathologic forms of primary endocardial fibroelastosis are dilated, which is most common, and contracted. (medscape.com)
  • citation needed] The term "endocardial fibroelastosis" was introduced by Weinberg and Himmelfarb in 1943. (wikipedia.org)
  • The term endocardial fibroelastosis was introduced by Weinberg and Himmelfarb in 1943. (medscape.com)
  • In 1943, the endocardial fibroelastosis term was coined by Weinberg et al. (nih.gov)
  • Viral infection of the myocardium in endocardial fibroelastosis. (medscape.com)
  • The endocardial thickening is believed to be caused by persistent and increased wall tension in the ventricles, possibly secondary to damaged myocardium, mitral regurgitation, or both. (medscape.com)
  • Fibroblasts in an endocardial fibroelastosis disease model mainly originate from mesenchymal derivatives of epicardium. (medscape.com)
  • A report suggests that the epicardium-derived mesenchymal cells could be the origin of the endocardial fibroelastosis fibroblasts. (medscape.com)
  • Secondary endocardial fibroelastosis is associated with other congenital heart diseases. (medscape.com)
  • Careful assessment of the heart for any associated congenital heart disease including coronary artery anomalies is essential before making a diagnosis of primary endocardial fibroelastosis. (medscape.com)
  • Risk of endocardial fibroelastosis in subsequent pregnancies is 3-5% and warrants fetal echocardiography for early diagnosis. (medscape.com)
  • We describe a case of very early diagnosis of fibroelastosis and aortic valve stenosis observed in utero at 14 weeks' gestation by transvaginal echocardiography. (nih.gov)
  • Primary endocardial fibroelastosis is not associated with any significant structural anomaly of the heart. (medscape.com)
  • Primary endocardial fibroelastosis prognosis is relatively poor, although the condition is not universally fatal. (medscape.com)
  • Primary endocardial fibroelastosis. (bmj.com)
  • Use endocardial fibroelastosis, malignant effusions. (mynarch.net)
  • Can send letters by osteoarthritis of movement, feel you have been lying between what can be congenital, mucopolysaccharidoses, endocardial fibroelastosis, malignant cells to be planning. (fountainheadapartmentsma.com)
  • Endocardial fibrosis principally involves the apices of the right and left ventricles and may affect the atrioventricular valves mainly by tethering the papillary muscles, leading to tricuspid and mitral regurgitation. (medscape.com)
  • https://www.barthsyndrome.org/bsf-international/Espanol Barth Syndrome Foundation - Espa�ol ~ Bienvenidos a la Fundaci�n del S�ndrome de Barth - Estamos dedicamos a salvar vidas por medio de educaci�n, avances en tratamiento, y la b�squeda de una cura para el s�ndrome de Barth. (spendlessformedicine.com)
  • Endocardial sentence examples:1.the relationship between the characteristic Endocardial electrogram and av node anterograde conduction during radiofrequency catheter ablation for atrioventricular nodal reentrant tachycardia2.objective to study a new computerized automatic method for Endocardial boundary estimation and tracking 120 sentence examples: 1. (netlify.app)
  • Keller SB, Cohen J, Moon-Grady A, Cuneo B, Paul E, Coll AC, Campbell M, Srivastava S. Patterns of endocardial fibroelastosis without atrioventricular block in fetuses exposed to anti-Ro/SSA antibodies. (ucdenver.edu)
  • Misdiagnosed anomalous left coronary artery from the pulmonary artery as endocardial fibroelastosis in infancy: A case series. (medscape.com)
  • Endocardial fibroelastosis has been associated with poor prognosis and outcomes. (nih.gov)
  • In 1957 Black-Schaffer proposed a unitary explanation that stress on the ventricle, of any kind, may trigger the endocardial reaction, so that all EFE could be thought of as secondary. (wikipedia.org)
  • Endocardial fibroelastosis (EFE) is primarily a disease of infants and children, but can rarely present in adulthood as well. (nih.gov)
  • Endocardial fibroelastosis (EFE), which occurs in infants and children, affects only the left ventricle. (msdmanuals.com)
  • Endocardial fibroelastosis (EFE) is a rare heart disorder usually occurring in children two years old and younger. (wikipedia.org)
  • Endocardial fibroelastosis of the left ventricle affects right ventricular performance in fetuses with hypoplastic left heart syndrome: a prospective study using M-mode, PW- and tissue Doppler techniques. (medscape.com)
  • Once regarded as a common cause of unexplained heart failure, endocardial fibroelastosis is now considered rare. (medscape.com)
  • Other signs and symptoms may include increased levels of certain organic acids in the urine and blood (such as 3-methylglutaconic acid), and increased thickness of the left ventricle of the heart due to endocardial fibroelastosis, which can cause potential heart issues. (nih.gov)
  • Endocardial fibroelastosis: a clinical and anatomic study of 47 patients with emphasis on its relationship to mitral insufficiency. (medscape.com)
  • EFE had quickly become the name of a disease, and it continues to be used by many physicians in this way, though many patients with identical symptoms do not have the endocardial reaction of EFE. (wikipedia.org)
  • MR findings of endocardial fibroelastosis in children. (medscape.com)
  • ventricular cavum is indicated by the red curve) and transmural homogeneous perfusion (no significant differences between the septal positions: endocardial layer indicated by the blue curve and outer layer by the yellow curve). (bmj.com)
  • Chen S, Thompson MW, Rose V. Endocardial fibroelastosis: family studies with special reference to counseling. (medscape.com)
  • On autopsy, most of these patients' hearts showed the thickened endocardial layer noted above. (wikipedia.org)
  • Despite the fact that generally sound, the Javanese is defenseless to endocardial fibroelastosis and bulge of the cranial sternum, a hereditary imperfection ordinarily found in varieties identified with the Siamese. (catsaesthetic.com)

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