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    a genetic disorder that reduces blood flow of human body

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    Sickle Cell Disease

    Sickle cell disease is a  blood disorder in which the hemoglobin is damaged and can't carry oxygen to the tissues. These blood cells with the defective hemoglobin are sticky and can build up and block small blood vessels leading to pain and other complications of the disease.

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    Sickle Cell Disease

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    What is sickle cell disease?

    Sickle cell disease is an inherited blood disorder. It is marked by flawed hemoglobin. That’s the protein in red blood cells that carries oxygen to the tissues of the body. So, sickle cell disease interferes with the delivery of oxygen to the tissues.

    Red blood cells with normal hemoglobin are smooth, disk-shaped, and flexible, like doughnuts without holes. They can move through the blood vessels easily. Cells with sickle cell hemoglobin are stiff and sticky. When they lose their oxygen, they form into the shape of a sickle or crescent, like the letter C. These cells stick together and can’t easily move through the blood vessels. This can block small blood vessels and the movement of healthy, normal oxygen-carrying blood. The blockage can cause pain.

    Normal red blood cells can live up to 120 days. But, sickle cells only live for about 10 to 20 days. Also, sickle cells may be destroyed by the spleen because of their shape and stiffness. The spleen helps filter the blood of infections. Sickled cells get stuck in this filter and die. With less healthy red blood cells circulating in the body, you can become chronically anemic. The sickled cells also damage the spleen. This puts you are at greater at risk for infections.

    What causes sickle cell disease?

    Sickle cell is an inherited disease caused by a defect in a gene.

    A person will be born with sickle cell disease only if two genes are inherited—one from the mother and one from the father.

    A person who inherits just one gene is healthy and said to be a "carrier" of the disease. A carrier has an increased chance of having a child with sickle cell disease if he or she has a child with another carrier.

    For parents who are each carriers of a sickle cell gene, there is a 1 in 4, or a 25 % chance of having a child with sickle cell disease.

    What are the risk factors for sickle cell disease?

    Having a family history of sickle cell disease increases your risk for the disease. In the United States, it mainly affects African Americans.

    What are the symptoms of sickle cell disease?

    The following is a list of symptoms and complications associated with sickle cell disease. However, each person may experience symptoms differently. Symptoms and complications may include:

    Anemia. Because sickled cells are short-lived or destroyed, there are less red blood cells available in the body. This results in anemia. Severe anemia can make you feel dizzy, short of breath, and tired.Pain crisis, or sickle crisis. This occurs when the flow of blood is blocked to an area because the sickled cells have become stuck in the blood vessel. The pain can occur anywhere, but most often occurs in the chest, arms, and legs. Infants and young children may have painful swelling of the fingers and toes. Interruption in blood flow may also cause tissue death.Acute chest syndrome. This occurs when sickling occurs in the chest. This can be life-threatening. It often occurs suddenly, when the body is under stress from infection, fever, or dehydration. The sickled cells stick together and block the flow of oxygen in the tiny vessels in the lungs. It resembles pneumonia and can include fever, pain, and a violent cough.Splenic sequestration (pooling). Crises are a result of sickle cells pooling in the spleen. This can cause a sudden drop in hemoglobin and can be life-threatening if not treated promptly. The spleen can also become enlarged and painful from the increase in blood volume. After repeated episodes,  the spleen becomes scarred, and permanently damaged. Most children, by age 8, do not have a working spleen either from surgical removal, or from repeated episodes of splenic sequestration. The risk of infection is a major concern of children without a working spleen. Infection is the major cause of death in children younger than age 5 in this population.Stroke. This is another sudden and severe complication of people with sickle cell disease. The misshapen cells can block the major blood vessels that supply the brain with oxygen. Any interruption in the flow of blood and oxygen to the brain can result in severe brain damage. If you have one stroke from sickle cell anemia, you are more likely to have a second and third stroke.Jaundice, or yellowing of the skin, eyes, and mouth. Jaundice is a common sign and symptom of sickle disease. Sickle cells do not live as long as normal red blood cells and, therefore, they are dying faster than the liver can filter them out. Bilirubin (which causes the yellow color) from these broken down cells builds up in the system causing jaundice.Priapism.  This is a painful obstruction of the blood vessels in the penis by sickle cells. If not promptly treated, it can result in impotence.

    The symptoms of sickle cell disease may look like other blood disorders or medical problems. Always consult your health care provider for a diagnosis.

    स्रोत : www.hopkinsmedicine.org

    Sickle cell disease: MedlinePlus Genetics

    Sickle cell disease is a group of disorders that affects hemoglobin , the molecule in red blood cells that delivers oxygen to cells throughout the body. Explore symptoms, inheritance, genetics of this condition.

    Sickle cell disease

    Sickle cell disease Description

    Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disease have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.

    Signs and symptoms of sickle cell disease usually begin in early childhood. Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain. The severity of symptoms varies from person to person. Some people have mild symptoms, while others are frequently hospitalized for more serious complications.

    The signs and symptoms of sickle cell disease are caused by the sickling of red blood cells. When red blood cells sickle, they break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs, such as the lungs, kidneys, spleen, and brain, of oxygen-rich blood and can lead to organ damage. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension), which can lead to heart failure. Pulmonary hypertension occurs in about 10 percent of adults with sickle cell disease.

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    The information on this site should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.

    स्रोत : medlineplus.gov

    Blood Disorder Types, Symptoms, and Treatments

    WebMD explains different types of blood disorders and their causes, symptoms, diagnosis, and treatment.

    CancerLeukemia & LymphomaReference

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    Types of Blood Disorders

    Written by Matthew Hoffman, MD

    Medically Reviewed by Jennifer Robinson, MD on March 17, 2021

    Blood disorders can affect any of the three main components of blood:

    Red blood cells, which carry oxygen to the body's tissues

    White blood cells, which fight infections

    Platelets, which help blood to clot

    Blood disorders can also affect the liquid portion of blood, called plasma.

    Treatments and prognosis for blood diseases vary, depending on the blood condition and its severity.

    Blood Disorders Affecting Red Blood Cells

    Blood disorders that affect red blood cells include:

    Anemia: People with anemia have a low number of red blood cells. Mild anemia often causes no symptoms. More severe anemia can cause fatigue, pale skin, and shortness of breath with exertion.

    Iron-deficiency anemia: Iron is necessary for the body to make red blood cells. Low iron intake and loss of blood due to menstruation are the most common causes of iron-deficiency anemia. It may also be caused by blood loss from the GI tract because of ulcers or cancer. Treatment includes iron pills, or rarely, blood transfusion.Anemia of chronic disease: People with chronic kidney disease or other chronic diseases tend to develop anemia. Anemia of chronic disease does not usually require treatment. Injections of a synthetic hormone, epoetin alfa (Epogen or Procrit), to stimulate the production of blood cells or blood transfusions may be necessary in some people with this form of anemia.Pernicious anemia (B12 deficiency): A condition that prevents the body from absorbing enough B12 in the diet. This can be caused by a weakened stomach lining or an autoimmune condition. Besides anemia, nerve damage (neuropathy) can eventually result. High doses of B12 prevent long-term problems.Aplastic anemia: In people with aplastic anemia, the bone marrow does not produce enough blood cells, including red blood cells. This can be caused by a host of conditions, including hepatitis, Epstein-Barr,  or HIV -- to the side effect of a drug, to chemotherapy medications, to pregnancy. Medications, blood transfusions, and even a bone marrow transplant may be required to treat aplastic anemia.Autoimmune hemolytic anemia: In people with this condition, an overactive immune system destroys the body's own red blood cells, causing anemia. Medicines that suppress the immune system, such as prednisone, may be required to stop the process.Thalassemia: This is a genetic form of anemia that mostly affects people of Mediterranean heritage. Most people have no symptoms and require no treatment. Others may need regular blood transfusions to relieve anemia symptoms.Sickle cell anemia: A genetic condition that affects mostly people whose families have come from Africa, South or Central America, the Caribbean islands, India, Saudi Arabia, and Mediterranean countries that include Turkey, Greece, and Italy. In sickle cell anemia, the red blood cells are sticky and stiff. They can block blood flow. Severe pain and organ damage can occur.Polycythemia vera: The body produces too many blood cells, from an unknown cause. The excess red blood cells usually create no problems but may cause blood clots in some people.Malaria: A mosquito's bite transmits a parasite into a person's blood, where it infects red blood cells. Periodically, the red blood cells rupture, causing fever, chills, and organ damage. This blood infection is most common in parts of Africa but can also be found in other tropical and subtropical areas around the world; those traveling to affected areas should take preventive measures.

    Blood Disorders Affecting White Blood Cells

    Blood disorders that affect white blood cells include:

    Lymphoma: A form of blood cancer that develops in the lymph system. In lymphoma, a white blood cell becomes malignant, multiplying and spreading abnormally. Hodgkin's lymphoma and non-Hodgkin's lymphoma are the two major groups of lymphoma. Treatment with chemotherapy and/or radiation can often extend life with lymphoma, and sometimes cure it.

    Leukemia: A form of blood cancer in which a white blood cell becomes malignant and multiplies inside bone marrow. Leukemia may be acute (rapid and severe) or chronic (slowly progressing). Chemotherapy and/or stem cell transplantation (bone marrow transplant) can be used to treat leukemia, and may result in a cure.

    Multiple myeloma: A blood cancer in which a white blood cell called a plasma cell becomes malignant. The plasma cells multiply and release damaging substances that eventually cause organ damage. Multiple myeloma has no cure, but stem cell transplant and/or chemotherapy can allow many people to live for years with the condition.Myelodysplastic syndrome: A family of blood cancers that affect the bone marrow. Myelodysplastic syndrome often progresses very slowly, but may suddenly transform into a severe leukemia. Treatments may include blood transfusions, chemotherapy and stem cell transplant.

    Blood Disorders Affecting Platelets

    Blood disorders that affect the platelets include:

    Thrombocytopenia : A low number of platelets in the blood; numerous conditions cause thrombocytopenia, but most do not result in abnormal bleeding.

    Idiopathic thrombocytopenic purpura: A condition causing a persistently low number of platelets in the blood, due to an unknown cause; usually, there are no symptoms, yet abnormal bruising, small red spots on the skin (petechiae), or abnormal bleeding can result.

    स्रोत : www.webmd.com

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