81. HealthDay Printer Friendly Format. Early Treatment Works With sickle cell disease Quick use of lifeprolonging drug may benefit patients, study found. http://www.healthday.com/view.cfm?id=518484

82. Bmj.com Claster And Vichinsky 327 (7424): 1151 Clinical review. Managing sickle cell disease. Advances are being made in the management of sickle cell disease for all age groups. http://bmj.bmjjournals.com/cgi/content/full/327/7424/1151

Home Help Search/Archive Feedback ... Table of Contents BMJ 2003;327:1151-1155 (15 November), doi:10.1136/bmj.327.7424.1151 PDF of this article Email this article to a friend Respond to this article Read responses to this article See related This week in BMJ item PubMed citation Related articles in PubMed Download to Citation Manager Citation Map Search Medline for articles by: Claster, S. Vichinsky, E. P Alert me when: New articles cite this article Collections under which this article appears: Genetics Haematology ( including blood transfusion) Clinical review Managing sickle cell disease Susan Claster chief of hematology clinics Elliott P Vichinsky director University of California at San Francisco, San Francisco General Hospital, 995 Portrero Avenue, Building 80, Ward 84, Box 0874, San Francisco, CA 94110, USA, Department of Hematology and Oncology, Children's Hospital and Research Center at Oakland, 747 52nd Street, Oakland 94609 CA, USA Correspondence to: E P Vichinsky Advances are being made in the management of sickle cell disease for all age groups. This review discusses the progress in amelioration

83. Action Medical Research - Sickle Cell Disease The situation sickle cell disease covers a group of inherited blood disorders which affect mainly AfroCaribbean people, as well as people in parts of Europe http://www.action.org.uk/news_media/sickle_cell.php

awmSelectedItem=6 news and media news press releases campaigns ... publications receive action alerts ! Sickle cell The situation Sickle cell disease covers a group of inherited blood disorders which affect mainly Afro-Caribbean people, as well as people in parts of Europe, South America and the Middle East. The most severe form of the disease affects one in 300-400 of the world's black population, and numbers are increasing in the UK. However, an opinion is being voiced in the UK that the amount of funding and attention the condition receives is influenced by another factor - the ethnic group affected by the illness. Racism? UK member of parliament Jane Griffiths, who represents Reading East, had no hesitation in suggesting that racial prejudice was holding up research into sickle cell disease when she spoke in a debate at the House of Commons early in the year 2000. Sickle cell disease, she pointed out, currently affects 10,000 people in the UK, compared with cystic fibrosis which affects 7,000 people. Both are genetic disorders, yet awareness and funding for cystic fibrosis is far greater than that of sickle cell disease.

84. BBC News | HEALTH | Gene Therapy Hope For Sickle Cell Disease Scientists have been able to correct sickle cell disease in mice using gene therapy. http://news.bbc.co.uk/hi/english/health/newsid_1708000/1708055.stm

CATEGORIES TV RADIO COMMUNICATE ... INDEX SEARCH You are in: Health Front Page World UK ... AudioVideo SERVICES Daily E-mail News Ticker Mobiles/PDAs Feedback ... Low Graphics Friday, 14 December, 2001, 14:24 GMT Gene therapy hope for sickle cell disease The sickle cell's distinctive shape Scientists have been able to correct sickle cell disease in mice using gene therapy. The American researchers who carried out the tests say there are several hurdles to be overcome before the therapy can be used in humans, but that it offers hope for the future. The research, detailed in the journal Science, shows how the gene therapy counteracts the faulty gene that causes red blood cells to "sickle", or become deformed. The treatment involves the "anti-sickling" variant being delivered into bone marrow; it then incorporates itself into the stem cells that give rise to red blood cells. 'Sticky' cells Sickle cell anaemia is an inherited blood disorder, which largely affects Afro-Caribbean and Asian patients, named after the distinctive shape formed by red blood cells. The shape is affected because a protein within the cells - haemoglobin which carries oxygen from the lungs to the rest of the body - is different to normal.

85. Exec Summary Vol. 4: No. 3. Screening For Sickle Cell Disease And Thalassaemia: suitable for printing). Screening for sickle cell disease and thalassaemia a systematic review with supplementary research. SC Davies 1 http://www.hta.nhsweb.nhs.uk/execsumm/summ403.htm

HTA Home Page Publications Page Health Technology Assessment 2000; Vol. 4: No. 3 (Executive summary) Executive summary View/Download full monograph in Adobe Acrobat format (731 kbytes) View/Download this 4-page summary in Adobe Acrobat format (suitable for printing) Screening for sickle cell disease and thalassaemia: a systematic review with supplementary research SC Davies E Cronin M Gill P Greengross M Hickman C Normand Imperial College School of Medicine, Central Middlesex Hospital, London, UK Department of Public Health, School of Hygiene and Tropical Medicine, London, UK Directorate of Public Health and Health Policy, Brent and Harrow Health Authority, Middlesex, UK Social Science and Medicine, Imperial College Medical School, London, UK Corresponding author Introduction The haemoglobinopathies (thalassaemias and sickle cell disease (SCD)) are inherited disorders of haemoglobin. In 1993, the UK Standing Medical Advisory Committee made the following recommendations. Preconceptual carrier diagnosis for these conditions should be encouraged. Antenatal and neonatal screening should be universal in districts where over 15% of the population are from ethnic minorities.

86. Sickle Cell Disorder Sickle Cell Disorder. sickle cell disease describes a group of inherited disorders of red blood cells. sickle cell disease affects more than 50,000 Americans. http://gslc.genetics.utah.edu/units/newborn/infosheets/sicklecelldisorder.cfm

Genetic Science Learning Center at the Eccles Institute of Human Genetics University of Utah Home ... Full Medical Reports Sickle Cell Disorder Sickle Cell Disorder Sickle cell disease describes a group of inherited disorders of red blood cells. Red blood cells are responsible for delivering oxygen to different parts of the body; normally they are round and contain a molecule called hemoglobin, which carries oxygen. If the gene encoding hemoglobin is mutated, it causes a change in the shape of the molecule. When the mutated hemoglobin delivers oxygen to the tissues, the red blood cell collapses, resulting in a long, flat sickle-shaped cell. These cells clog blood flow, resulting in a variety of symptoms including pain, increased infections, lung blockage, kidney damage, delayed growth and anemia (low blood cell count). Genetics The gene encoding the beta chain of the hemoglobin molecule, located on chromosome 11, can be mutated in a variety of ways that result in different types of sickle cell disease. Some mutations are more common than others. The three most common types of sickle cell disease in the United States are hemoglobin SS (Hb SS), hemoglobin SC (Hb SC), and hemoglobin sickle beta-thalassemia (HbS beta-thalassemia). Inheritance Autosomal recessive.

87. THE MERCK MANUALSECOND HOME EDITION, Sickle Cell Disease In Ch. sickle cell disease. sickle cell disease is an inherited sickle cell disease affects blacks almost exclusively. About 10% of blacks in the http://www.merck.com/mrkshared/mmanual_home2/sec14/ch172/ch172g.jsp

88. HeartCenterOnline For Patients - Doppler Recommended For Sickle Cell Disease Doppler recommended for sickle cell disease, advertisement. Mar 03 (HeartCenterOnline) As many as one out of every three adults http://www.heartcenteronline.com/myheartdr/home/research-detail.cfm?reutersid=41

89. Anemia, Sickle Cell Management of sickle cell disease. Guidelines are available for various health professionals on haemoglobin types, sickle cell disease, and the thalassaemias. http://omni.ac.uk/browse/mesh/C0002895L0002895.html

low graphics Anemia, Sickle Cell other: Thalassemia narrower: Sickle Cell Trait Sickle Cell Information Center This site aims to provide information to patients and health care professionals on the topic of sickle cell anaemia and also offers a collection of resources on sickle cell for patients, family carers, students and teachers. It is developed and maintained by its advisory board which is made up of the main partner institutions like the Emory University School of Medicine and the Sickle Cell Foundation of Georgia, and states its mission as being to "provide sickle cell patient and professional education, news, research updates and world wide sickle cell resources". It makes available online articles, powerpoint demonstrations, links to books, videos and other useful Web sites. Anemia, Sickle Cell Management of sickle cell disease This is the full-text of a report on the management of sickle cell disease, produced by the National Heart, Lung and Blood Instutute (NHLBI), of the National Institutes of Health (NIH) in the US. It is a clinical guide aimed at health care professionals, and describes the current approach to counseling and also to management of many of the medical complications of sickle cell disease. It is in four sections: Diagnosis and Counseling, Health Maintenance, Treatment of Acute and Chronic Complications, and Special Topics. The document is PDF, which requires the Adobe Acrobat Reader. Updated in May 2002. Sickle Cell Trait Anemia, Sickle Cell

90. SICKLE CELL DISEASE sickle cell disease. sickle cell disease is a worldwide health problem affecting many races, countries, and ethnic groups. It is http://www.utmbhealthcare.org/hil/GENE4594.asp?header=

91. The Scientist - Murine Gene Therapy Corrects Symptoms Of Sickle Cell Disease RESEARCH. Murine Gene Therapy Corrects Symptoms of sickle cell disease. FACULTY of 1000 Early results indicate that human trials are not far off. Email article. http://www.the-scientist.com/yr2002/mar/research3_020318.html

The Scientist 16[6]:36, Mar. 18, 2002 RESEARCH Murine Gene Therapy Corrects Symptoms of Sickle Cell Disease E-mail article By Jennifer Fisher Wilson FACULTY OF 1000 The Faculty of 1000 is a Web-based literature awareness tool published by BioMed Central. It provides a continuously updated insider's guide to the most important peer-reviewed papers within a range of research fields, based on the recommendations of a faculty of more than 1,400 leading researchers. Each issue, The Scientist publishes a list of the 10 top-rated papers from a specific subject area, as well as a short review of one or more of the listed papers. We also publish a selection of comments on interesting recent papers from the Faculty of 1000's output. For more information visit www.facultyof1000.com Caused by a simple gene mutation that misshapes red blood cells and renders them ineffective, sickle cell disease (SCD) seemed to provide scientists with a straightforward target for gene therapy. But since the first SCD gene therapy experiment in 1979, researchers continue to struggle. It wasn't until late 2001, in a paper listed in the Faculty of 1000's Top 10 Genomics list for Feb. 15, that scientists corrected the murine symptoms of this chronic, inherited, and often painful disorder. F1000 Top 10 All of Biology 1. A.-C. Gavin et al., "

92. American Red Cross : Sickle Cell Disease Donations Be an African American Hero to a Child with sickle cell disease Through a oneof-a-kind program, the American Red Cross and The Children s Hospital of http://www.pleasegiveblood.org/donating/sickle_cell.php

Search this site Click Here to View Thank You Cards... Be an African American Hero to a Child with Sickle Cell Disease Through a one-of-a-kind program, the American Red Cross and The Children's Hospital of Philadelphia are improving care for children with sickle cell disease - and we need your help! Sickle cell disease is an inherited disease that affects more than 70,000 people in the United States, ninety percent of whom are of African descent. According to Kim Smith-Whitley, MD, of The Children's Hospital of Philadelphia, sickle cell disease causes anemia; jaundice; damage to the lungs, kidney and spleen; acute pain; and strokes. Sickle cell disease patients, especially young children, may be easily overwhelmed by infections. There is no cure for sickle cell disease, but good medical care, including blood transfusions, can help manage and prevent the pain and serious complications of the disease. Blood Donors Are Heroes Blood donors are the real heroes in this story because some patients with sickle cell disease need many units of blood every month! But if the blood is not a very close match, the patient may reject the blood and a source of help and hope will be gone. This condition, called alloimmunization, can be fatal. The best matches for these patients will come from African American donors. African American donors who want to help can choose to give blood for a child with sickle cell disease by using a special blue tag available at all Red Cross blood drives and donor centers in the Greater Delaware Valley and New Jersey.

93. PULMONARY COMPLICATIONS OF SICKLE CELL DISEASE Fund number RFAHL-04-015 Title PULMONARY COMPLICATIONS OF sickle cell disease Grant - Posted on Jan 20, 2004. SEARCH for Grants. http://www.fedgrants.gov/Applicants/HHS/NIH/NIH/RFA-HL-04-015/listing.html

Department of Health and Human Services Grant Opportunities Office : National Institutes of Health Location : National Institutes of Health Fund number : RFA-HL-04-015 Title : PULMONARY COMPLICATIONS OF SICKLE CELL DISEASE Grant - Posted on Jan 20, 2004 SEARCH for Grants

94. Dr. Koop-Major Complication Of Sickle Cell Disease Found Major Complication of sickle cell disease Found. sickle cell disease is a genetic disease that occurs predominantly in people of African descent. http://www.drkoop.com/template.asp?ap=93&page=newsdetail&id=8008091

95. Children's Hemiplegia And Stroke Association Children with sickle cell disease are 300 times more likely than normal children to have a stroke. sickle cell disease The neurological complications May 2002. http://www.chasa.org/sicklecell.htm

Home About CHASA Support Newly Diagnosed ... Contact CHASA sickle cell and stroke S ickle cell anemia is an inherited, chronic disease in which the red blood cells, normally disc-shaped, become crescent shaped. As a result, they function abnormally and break down, causing recurrent painful episodes. Sickle cell anemia is caused by an abnormal type of hemoglobin (oxygen carrying pigment) called hemoglobin S. It is inherited as an autosomal recessive trait; that is, it occurs in someone who has inherited hemoglobin S from both parents. From the medical college of Georgia (1999): "Seventeen percent of all SCD patients suffer a stroke. Of all people with sickle cell anemia, children have the highest stroke risk. Their risk is greatest between ages of five and sixteen. Children with sickle cell disease are 300 times more likely than normal children to have a stroke. Of the children who had a stroke and survived, 70% will have a second stroke and it usually occurs within 36 months of the first stroke." Stroke in children and sickle-cell disease Baltimore-Washington Cooperative Young Stroke Study The Sickle Cell Information Center Emory University School of Medicine Brain imaging findings in pediatric patients with sickle cell disease . July 2003. Tennessee. Stroke risk in siblings with sickle cell anemia , March 2003, multicenter study.

96. Newborn Screening Program - Sickle Cell Disease And Other Hemoglobinopathies sickle cell disease and Other Hemoglobinopathies. sickle cell disease and other sickling hemoglobin disorders are inherited in an autosomal recessive pattern. http://www.idph.state.il.us/HealthWellness/fs/sickle.htm

Sickle Cell Disease and Other Hemoglobinopathies Definition Sickling hemoglobinopathies are inherited disorders that result in production of an abnormal form of hemoglobin S, C or E, or a decreased synthesis of a beta globin chain. Sickle cell disease is a term for hemoglobin disorders characterized by the predominate production of hemoglobin S. Clinical Symptoms Any sign of illness in an infant with sickling disease is a potential medical emergency. Acute and chronic tissue injury can occur when sickled cells cause vascular occlusion. Sickling diseases can cause severe pain anywhere in the body, but most often in the hands, arms, chest, legs and feet. Complications may include, but are not limited to, the following: Newborn Screening and Definitive Diagnosis There are several recommended testing methods for diagnosis of sickling disorders and hemoglobinopathies: Hemoglobin electrophoresis including both cellulose acetate and citrate agars (one is not sufficient), isoelectric focusing and high performance liquid chromatography are considered proven, reliable and accurate methods for defining an infant's hemoglobin phenotype. All siblings of infants diagnosed with sickle cell disease or other serious hemoglobinopathies should be tested; genetic counseling services should be offered to parents. Treatment The National Institutes of Health clinical guidelines for management of sickle cell disease state, "Penicillin prophylaxis should begin by 2 months of age for infants with suspected sickle cell anemia, whether or not the definitive diagnosis has been established." Antibiotic therapy should continue until at least 5 years of age. Normal dosage for an infant is 125 mg of penicillin twice a day until 3 years of age, when dosage is increased to 250 mg twice a day. An alternative antibiotic is available for children who are allergic to penicillin therapy. Prescription pain medication also may be indicated during sickling crises. Health care monitoring and maintenance with appropriate immunizations is imperative to the health of the baby, and pneumococcal conjugate vaccine immunizations also are recommended, beginning at 2 months of age.

97. High-Risk Pregnancy - Sickle Cell Disease sickle cell disease. What is sickle cell disease? sickle cell disease involves the red blood cells, or hemoglobin, and their ability to carry oxygen. http://www.chkd.org/High_Risk_Pregnancy/sickcell.asp

More Health Information Adolescent Medicine Allergy/Immunology Anesthesiology Arthritis Burns Cardiology Craniofacial Dental Medicine Dermatology Developmental Peds Diabetes Digestive Ear, Nose, Throat Genetics Gastroenterology Growth Hematology High Risk Newborn High Risk Pregnancy Infectious Disease Mental Health Neonatology Nephrology Neurology Normal Newborn Normal Pregnancy Oncology Ophthalmology Orthopedics Otolaryngology Pediatric Intensive Care Pediatric Surgery Pediatrics Physical Medicine Plastic Surgery Respiratory/Pulmonology Rheumatology Safety Surgery Terminal Transplant Urology Site Search Sickle Cell Disease What is sickle cell disease? Sickle cell disease is an inherited blood disorder characterized by defective hemoglobin (a protein inside red blood cells that carries oxygen to the tissues of the body). Sickle cell disease involves the red blood cells, or hemoglobin, and their ability to carry oxygen. Normal hemoglobin cells are smooth, round, and flexible, like the letter “O,” so they can move through the vessels in our bodies easily. Sickle cell hemoglobin cells are stiff and sticky, and form into the shape of a sickle, or the letter “C,” when they lose their oxygen. These sickle cells tend to cluster together, and cannot easily move through the blood vessels. The cluster causes a blockage and stops the movement of healthy, normal oxygen-carrying blood. This blockage is what causes the painful and damaging complications of sickle cell disease.

98. NORD - National Organization For Rare Disorders, Inc. sickle cell disease. To Sickle CellThalassemia Disease. General Discussion sickle cell disease is a rare inherited blood disorder. It http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Sickle Cell D

99. Sickle Cell Disease Follow-up Program sickle cell disease Follow up Program. However, the state currently lacks a comprehensive program for adults with sickle cell disease. http://www.fha.state.md.us/genetics/html/sickle_pro.html

Center for Cancer Surveillance and Control Genetics and Children Center for Maternal and Child Health Center for Preventive Health Services Child and Adolescent Health Health Promotion, Education Primary Care And Rural Health Oral Health Deer's Head Center Western Maryland Center GENETICS HOME Newborn Screening Sickle Cell Disease Metabolic Disorders ... Children's Medical Services and Regional Resource Development Sickle Cell Disease Follow up Program This program follows children with sickle cell disease and other hemoglobin disorders long term, with the following services: providing case management, home visiting, annual pediatric hematology evaluations (outreach clinics), genetic counseling, parent education, support groups and summer camp. Maryland has an excellent program for young children with sickle cell disease coordinated by the Office for Genetics and Children with Special Health Care Needs. A recent study* shows that the mortality among young children with sickle cell disease in Maryland is the lowest in the US. However, the state currently lacks a comprehensive program for adults with sickle cell disease. Changes in the pattern of health care delivery are placing most patients in managed care increasing the importance of following available national treatment guidelines.

100. Sickle Cell Disorder - For Patients And Families - The Center For Human Genetics Duke Comprehensive Sickle Cell Center conduct a NIH research study to determine if inherited factors relate to specific complications of sickle cell disease. http://www.chg.duke.edu/patients/sickle.html

For Patients and Families Sickle Cell Disorder (SCD) What is Sickle Cell Disease? Managing Sickle Cell Health Problems Resources for Managing Sickle Cell Disease What is Sickle Cell Disease? Sickle Cell Disease is a group of inherited disorders (passed from parents to their child) that affect the hemoglobin protein found in red blood cells. The hemoglobin that is found in individuals with Sickle Cell Disease causes the red blood cells to be hard, sticky, and shaped like a sickle (like a crooked banana). Normal red blood cells are soft, smooth, round and last about 120 days. Sickle red blood cells only last about 20 days. Usually, individuals receive two copies of the hemoglobin A gene, one from each parent. Individuals with Sickle Cell Disease do not have any copies of the hemoglobin A gene. Instead, these individuals have received two copies of an alternative form of the hemoglobin gene. At least one of those two alternative genes must be hemoglobin S in order to result in Sickle Cell Disease. The following section describes the various types of sickle cell disease and what genes those individuals carry. Types of Sickle Cell Disease There are three main types of sickle cell disease: Hemoglobin SS disease: Individuals have received two copies of the hemoglobin S gene.

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