61. Sickle Cell Disease sickle cell disease describes a group of inherited blood disorders characterized sickle cell disease Program, Division of Blood Diseases and Resources. http://www.healthatoz.com/healthatoz/Atoz/ency/sickle_cell_disease.jsp

62. GeneReviews: Sickle Cell Disease Your browser does not support HTML frames so you must view sickle cell disease in a slightly less readable form. Please follow this link to do so. http://www.genetests.org/query?dz=sickle

63. Health & Medical > Sickle Cell Disease Learn more about sickle cell disease in the following articles written by experts in the General Medical Care for Children With sickle cell disease http://www.drspock.com/topic/0,1504,467,00.html

September 09, 2005 SEARCH drSpock my account sign in HOT TOPICS Pregnancy Symptoms Read with Your Kids It's Fun! Take Our Quizzes Play with Your Baby TOPICS Parents are talking about their children. Join the discussion. Sickle Cell Disease The Dr. Spock Company and the Discovery Health Channel reveal the human face of a little understood genetic disorder in the television special Sickle Cell: The Forgotten Disease (check your local TV listings for dates and times). Learn more about sickle cell disease in the following articles written by experts in the field. Sickle Cell Disease Topics Sickle Cell: The Forgotten Disease Puts a Human Face on a Little Understood Genetic Disorder Sickle Cell Disease: The Basics The Subtle Differences of Sickle Cell Syndromes ... Treating Sickle Cell Disease with Bone Marrow Transplantation Questions From Other Parents The Lifespan for a Person with Sickle Cell Disease Lack of Emphasis on Sickle Cell Disease How Sickle Cell Disease Is Inherited Treating Children with Sickle Cell Disease ... Ensuring that a Child Won't Have Sickle Cell CONSUMER ALERTS CPSC Warns: Summer Fun Brings More Emergency Room Visits CPSC, K'NEX Industries Announce Recall of Children's Toys

64. Anemia, Sickle Cell sickle cell disease in childhood Part I. Laboratory diagnosis, pathophysiology and New Views of sickle cell disease Pathophysiology and Treatment. http://www.5mcc.com/Assets/SUMMARY/TP0056.html

Anemia, sickle cell DESCRIPTION: A chronic hemoglobinopathy transmitted genetically, marked by moderately severe chronic hemolytic anemia, periodic acute episodes of painful "crises," and increased susceptibility to intercurrent infections, especially S. pneumoniae. The heterozygous condition (Hb A/S) is called sickle cell trait and is usually asymptomatic, with no anemia. System(s) affected: Hemic/Lymphatic/Immunologic, Musculoskeletal Genetics: Autosomal recessive, mostly in blacks. Homozygous presence of a variant hemoglobin, HbS or sickle hemoglobin. Heterozygous condition Hb A/S. Incidence/Prevalence in USA: Approximately 1/500 black Americans and 1/1000 Hispanics have sickle cell anemia; 10% black Americans have sickle trait Predominant age: All ages Predominant sex: Male = Female CAUSES: At molecular level: Hb S is produced by substitution of valine for glutamic acid in the sixth amino acid position of the beta chains of the hemoglobin molecule. When deoxygenated, Hb S polymerizes and forms long rods that change RBC from biconcave to sickle shape. At cellular level: Sickle RBCs are inflexible; odd shape and cell rigidity cause increased blood viscosity, stasis, and mechanical obstruction of small arterioles and capillaries, leading to distal ischemia. Sickle RBCs are also more fragile than normal, leading to hemolytic destruction in blood and reticuloendothelial system.

65. Sickle Cell Disease -- Buchanan Et Al. 2004 (1): 35 -- Hematology DRUG TREATMENT FOR sickle cell disease THE OLD AND THE NEW. Martin H. Steinberg, MD*. Treatment of sickle cell disease Is Based on Pathophysiology http://www.asheducationbook.org/cgi/content/full/2004/1/35

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This Article Abstract Full Text (PDF) Citation Map Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager PubMed PubMed Citation Articles by Buchanan, G. R. Articles by Steinberg, M. H. Hematology 2004 The American Society of Hematology Sickle Cell Disease George R. Buchanan Michael R. DeBaun Charles T. Quinn and Martin H. Steinberg Abstract Much progress has been made during the past several decades in gaining understanding about the natural history of sickle cell disease and management approaches aimed at treating or even preventing certain disease complications. The characterization of the human genome now offers the opportunity to understand relationships regarding how gene polymorphisms as well as how environmental factors affect the sickle cell disease phenotype, as well as their specific organ function. This chapter explores some of these recent advances in knowledge.

66. Sickle Cell Disease -- Topic Overview What is sickle cell disease? sickle cell disease is an inherited blood disorder that turns normal, round http://my.webmd.com/hw/health_guide_atoz/hw254176.asp

var guid_source = ""; var guid_source_id = ""; //unused var encodedurl = ""; WebMD Today Home WebMD News Center XML News via RSS Member Services WebMD University My WebMD Find a Physician Medical Info Check Symptoms Medical Library Health Tools Clinical Trials ... Women, Men, Lifestyle Who We Are About WebMD Site Map Health Topics Symptoms ... Credits Sickle Cell Disease Topic Overview What is sickle cell disease? Sickle cell disease is an inherited blood disorder that turns normal, round blood cells into misshaped cells that look like sickles or crescent moons. These sickled cells can get stuck in blood vessels, blocking blood flow and causing severe pain as well as damage to organs, muscles, and bones. Complications of sickle cell disease can include: Painful events (painful crises), caused when blood flow is blocked by sickled cells. Acute chest syndrome, a possibly life-threatening condition. Splenic sequestration, caused by large numbers of blood cells trapped in the spleen. Anemia Infection.

67. ClinicalTrials.gov - Information On Clinical Trials And Human Research Studies: Conditions Graft Versus Host Disease; Sickle Cell Anemia; Thalassemia Conditions sickle cell disease; Iron Overload; Hemolytic Anemia http://www.clinicaltrials.gov/ct/gui/action/FindCondition?ui=D000755&recruiting=

68. Sickle Cell Anemia Article describes sickle cell disease, its diagnosis, and treatment. http://rarediseases.about.com/cs/sicklecell/a/110202.htm

var zLb=12; var zIoa1 = new Array('Suggested Reading','Sickle Cell Anemia Treatments','http://rarediseases.about.com/cs/sicklecell/a/110602.htm','Internet links on sickle cell disease','http://rarediseases.about.com/cs/sicklecell/'); var zIoa2 = new Array('Elsewhere on the Web','American Sickle Cell Anemia Association: Sickle Cell FAQs','http://www.ascaa.org/what_is.htm','NHLBI: Facts About Sickle Cell Anemia','http://www.nhlbi.nih.gov/health/public/blood/sickle/sca_fact.htm'); zJs=10 zJs=11 zJs=12 zJs=13 zc(5,'jsc',zJs,9999999,'') zfs=0;zCMt='a70' About Rare / Orphan Diseases Information by Disease Type Blood Disorders Sickle Cell Anemia Rare Diseases Essentials Rare Diseases: Basic Information Rare Diseases Support Groups ... Help zau(256,140,140,'el','http://z.about.com/0/ip/417/C.htm','');w(xb+xb+' ');zau(256,140,140,'von','http://z.about.com/0/ip/496/7.htm','');w(xb+xb); FREE Newsletter Sign Up Now for the Rare / Orphan Diseases newsletter! See Online Courses Search Rare / Orphan Diseases Stay up to date! Compare Prices Email to a friend ... Print this page Suggested Reading Sickle Cell Anemia Treatments Internet links on sickle cell disease Elsewhere on the Web American Sickle Cell Anemia Association: Sickle Cell FAQs NHLBI: Facts About Sickle Cell Anemia Most Popular Muscular Dystrophy ALD and Lorenzo's Oil Progeria Syndromes Leprosy (Hansen's Disease) ... The Elephant Man What's Hot Charcot-Marie-Tooth Disease Peyronie's Disease Twin-to-Twin Transfusion Synd Autoimmune Kidney Disease ... Cri-du-Chat Syndrome adunitCM(150,100,'x55')

69. Noah.cuny.edu/pregnancy/march_of_dimes/birth_defec American Red Cross sickle cell disease DonationsBe an African American Hero to a Child with sickle cell disease sickle cell disease patients, especially young children, may be easily overwhelmed by http://noah.cuny.edu/pregnancy/march_of_dimes/birth_defects/siklcell.html

70. HUM-MOLGEN: Discovery Promises Simpler Therapy For Sickle Cell Disease news section of the international communication forum in human genetics hummolgen. http://hum-molgen.org/NewsGen/02-2005/msg09.html

home genetic news bioinformatics biotechnology ... register for news alert Discovery Promises Simpler Therapy For Sickle Cell Disease February, 8 2005 8:13 Durham, N.C. – A new understanding of the causes for symptoms of sickle cell disease, a condition affecting one in every 600 African-Americans, has resulted from a study by researchers at Duke University Medical Center and Howard Hughes Medical Institute (HHMI). Their findings may lead to a new, more direct method for treating the disease, they said. Their research suggests that an inability of red blood cells to relax blood vessels through the release of nitric oxide is a major factor behind the disease's primary symptoms including oxygen deprivation and blocked vessels that can lead to pain, clots and stroke. Thus, therapies that restore nitric oxide to blood cells might serve as a useful method for treating the disease, said HHMI researcher Jonathan Stamler, M.D., professor of pulmonary medicine and cardiology at Duke University Medical Center. The researchers reported their findings on Jan. 31, 2005, in an early edition of Proceedings of the National Academy of Sciences.

71. New Advances In Sickle Cell Disease ? Excruciating pain episodes, recurrent pneumonias, strokes, severe infections, chronic hemolytic anemia, and secondary pulmonary hypertension are common http://www.news-medical.net/?id=6720

72. Sickle Cell Disease sickle cell disease is a genetically determined disorder of the red blood cell in which the protein structure of the ß subunit of the hemoglobin molecule is http://www.neuro.mcg.edu/cvhp/ss.html

Introduction Stroke Sickle Cell Disease The Circle of Willis Transcranial Dopler Ultrasound Stroke Prevention in Sickle Cell Study (STOP) Project S ICKLE C ELL D ISEASE Sickle cell disease is a genetically determined disorder of the red blood cell in which the protein structure of the ß subunit of the hemoglobin molecule is abnormal. A single base pair substitution in codon 6 causes the amino acid valine to be switched for glutamate. Under conditions of low oxygen tension, the abnormal hemoglobin precipitates. The erythrocyte deforms, assuming a sickle shape. The sickled cells do not circulate well; the hemoglobin polymerizes(if deoxygenated), leading to anemia, tissue oxygen starvation and infarction. Clinically, the patient suffers painful crises. Medical College of Georgia Neurology Department Home Page Medical College of Georgia Please email comments, suggestions, or questions to: dwloring@neuro.mcg.edu August 1999

73. THE MERCK MANUAL--SECOND HOME EDITION, Sickle Cell Disease In Ch. 172, Anemia In sickle cell disease, the red blood cells contain an abnormal form of People who have sickle cell disease always have some degree of anemia and mild http://www.merck.com/mmhe/sec14/ch172/ch172g.html

var externalLinkWarning = "The link you have selected will take you to a site outside Merck and The Merck Manuals.*n*nThe Merck Manuals do not review or control the content of any non-Merck site. The Merck Manuals do not endorse and are not responsible for the accuracy, content, practices, or standards of any non-Merck sources."; Search The Second Home Edition , Online Version Search Index A B C D ... Z Sections Accidents and Injuries Blood Disorders Bone, Joint, and Muscle Disorders Brain, Spinal Cord, and Nerve Disorders ... Women's Health Issues Resources Anatomical Drawings Multimedia Pronunciations Weights and Measures ... , Online Version Section Blood Disorders Chapter Anemia Topics Introduction Anemia From Excessive Bleeding Anemia of Chronic Disease Autoimmune Hemolytic Anemia ... Iron Deficiency Anemia Sickle Cell Disease Thalassemias Vitamin Deficiency Anemia Sickle Cell Disease Buy The Book Print This Topic Email This Topic Pronunciations amniocentesis coccal electrophoresis elliptocytosis ... thalassemia Sickle cell disease is an inherited condition characterized by sickle (crescent)-shaped red blood cells and chronic anemia caused by excessive destruction of red blood cells. Sickle cell disease affects blacks almost exclusively. About 10% of blacks in the United States have one copy of the gene for sickle cell disease (that is, they have sickle cell trait); they do not develop sickle cell disease, although rarely they may notice blood in their urine. About 0.3% of blacks have two copies of the gene; they develop the disease.

74. African American Women: Sickle Cell Anemia Pain Assessment and Pain Management in sickle cell disease A Guidebook for sickle cell disease From the March of Dimes, this publication answers http://www.4woman.gov/minority/aasickle.cfm

Skip navigation Health Problems in African American Women: Sickle Cell Anemia Sickle cell anemia (sih-kul sell uh-nee-mee-uh) is a blood disorder, passed down from parents to children. It involves problems the red blood cells. Normal red blood cells are round and smooth and move through blood vessels easily. Sickle cells are hard and have a curved edge. These cells cannot squeeze through small blood vessels. They block the organs from getting blood. Your body destroys sickle red cells quickly, but it can't make new red blood cells fast enough a condition called anemia. Sickle cell anemia can cause serious health problems: pain and swelling the hands and feet fatigue shortness of breath pain in any organ or joint eye damage yellow color in skin and eyes slow to grow hard to fight infections stroke chest pain fever Every year, about 1 in 600 African Americans are born with sickle cell disease. This means that they got the sickle gene from both parents. People who have only one gene are carriers of the sickle cell, but won't get sickle cell anemia themselves. They can pass the gene to their children. It is estimated that one in 12 African Americans have one sickle cell gene. Last updated: January 2005 E-mail this page to a friend Publications - This booklet is a review of pain prevention, home treatment, emergency treatment, and inpatient treatment in the hospital. The more one knows about the causes, prevention and treatment of pain, the better the chances of an early recovery. There are different types and different causes of pain requiring a clinician’s help and advice about treatment.

75. Sickle Cell Disease: Information From Answers.com sickle cell disease (Sickle Cell Anemia) What is sickle cell anemia? Sickle cell anemia (sickle cell disease) is a disorder of the blood caused by an. http://www.answers.com/topic/sickle-cell-anaemia

showHide_TellMeAbout2('false'); Business Entertainment Games Health ... More... On this page: Diagnosis Encyclopedia WordNet Best of Web Mentioned In Or search: - The Web - Images - News - Blogs - Shopping sickle cell disease Diagnosis Sickle Cell Disease (Sickle Cell Anemia) What is sickle cell anemia? Sickle cell anemia (sickle cell disease) is a disorder of the blood caused by an inherited abnormal hemoglobin (an oxygen-carrying protein within the red blood cells). The abnormal hemoglobin causes distorted (sickled) red blood cells. The sickled red blood cells are fragile and prone to rupture. When the number of red blood cells decreases from rupture (hemolysis), anemia is the result. This condition is referred to as sickle cell anemia. The irregular sickled cells can also block blood vessels causing tissue and organ damage and pain. Sickle cell anemia is one of the most common inherited blood anemias. The disease primarily affects Africans and African Americans. It is estimated that in the United States, some 50,000 African Americans are afflicted with the most severe form of sickle cell anemia. Overall, current estimates are that one in 1,875 U.S. African American is affected with sickle cell anemia. How is sickle cell anemia inherited?

76. Sickle Cell Disease - Disease Information Health information discussing sickle cell disease, which is a hereditary blood disorder that affects the red blood cell. http://www.mckinley.uiuc.edu/health-info/dis-cond/misc/sicklecd.html

Go to PDF Version Sickle Cell Disease WHAT IS SICKLE CELL DISEASE? Sickle Cell Disease is a hereditary blood disorder that affects the oxygen-carrying component of blood, the red blood cell. In other words, it is manifested by diseased red blood cells that have a sickle shape. Red blood cells have proteins called hemoglobin. Hemoglobin transports oxygen from your lungs to every part of your body. When a normal red blood cell (with normal hemoglobin) releases its oxygen, it maintains its discoid shape. However, when a diseased red blood cell containing sickle hemoglobin releases its oxygen, the appearance of the cell can change from discoid to sickle-shaped. This is to say that the sickle cell shape is caused by the sickle hemoglobin within a particular red blood cell. Possible health problems may include fatigue, breathlessness, rapid heartbeat, delayed growth, susceptibility to infections, skin ulcers, and vision problems. NORMAL HEMOGLOBIN vs. SICKLE HEMOGLOBIN While sickle hemoglobin and normal hemoglobin carry the same amount of oxygen, two major differences exist between the two kinds of cells. First, they differ in the way they flow through the blood vessels. Normal hemoglobin is found in disc-shaped red blood cells that are soft (like a bag of jelly), which enables them to easily flow through small blood vessels. Diseased red blood cells, which are sickle-shaped and are hard (like pieces of wood), often get stuck in small blood vessels and stop the flow of blood.

77. Sickle Cell Anemia Sickle cell anemia. Anemia, sickle cell. Hemoglobin S disease sickle cell disease Peripheral blood smear showing sickle cells admixed with target red http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=270

78. Clinical Practice Guidelines sickle cell disease is caused by structurally abnormal haemoglobin (Hb S) that Patients with sickle cell disease may present with the following problems http://www.rch.org.au/clinicalguide/cpg.cfm?doc_id=5245

79. THE GEOGRAPHY OF SICKLE CELL DISEASE: OPPORTUNITIES FOR UNDERSTANDING ITS DIVERS Homozygous sickle cell disease inheritance of the gene for HbS from both The distribution of the genotypes of sickle cell disease depends on the http://www.kfshrc.edu.sa/annals/143/rev9239.html

May 1994 THE GEOGRAPHY OF SICKLE CELL DISEASE: OPPORTUNITIES FOR UNDERSTANDING ITS DIVERSITY Graham R. Serjeant, MD, FRCP Address reprint requests and correspondence to Dr. Serjeant: MRC Laboratories (Jamaica), University of the West Indies, Kingston 7, Jamaica, West Indies. Accepted for publication 27 November 1993. The sickle cell gene is now known to be widespread, reaching its highest incidence in equatorial Africa, but occurring also in parts of Sicily and Southern Italy, Northern Greece, Southern Turkey, the Middle East, Saudi Arabia, especially the Eastern Province, and much of Central India (Figure 1). This distribution is determined by the occurrence of the sickle cell mutation and its selection by falciparum malaria. Studies of the structure of DNA surrounding the beta globin locus reveal that the sickle cell gene is associated with several DNA structures probably representing different ancestral populations. The most likely interpretation is that the sickle cell mutation is a relatively recent occurrence that has occurred independently in several different populations. Falciparum malaria then acted as a selective factor, increasing the prevalence of the gene because people inheriting the sickle cell gene from one parent and a gene for normal adult hemoglobin from the other parent (sickle cell trait) were less likely to die from malaria and so more likely to survive and pass on their genes. Over the generations, the sickle cell trait has therefore reached high frequencies in malarious areas. The factor in common to the distribution of the sickle cell gene is therefore malaria and not African ancestry.

80. Handbook Of Ocular Disease Management - Sickle Cell Disease In the four variations of sickle cell disease, systemic and ocular tissues With respect to the eye, the sickle cell disease mutation (SC) produces the http://www.revoptom.com/handbook/sect7e.htm

SICKLE CELL DISEASE SIGNS AND SYMPTOMS Ocular symptoms are uncommon in the early stages of the disease. Systemically, symptoms include painful crises of abdominal and musculoskeletal discomfort. Ocular signs include comma-shaped vessels in the bulbar conjunctiva, iris atrophy, iris neovascularization, dull-gray fundus appearance, retinal venous tortuosity, nonproliferative retinal hemorrhages in the subretinal, intraretinal or preretinal position. Further signs include black sunbursts (retinal pigment epithelial hyperplasia secondary to deep retinal vascular occlusions), glistening refractile deposits in the retinal periphery (hemosiderin-laden macrophages), salmon patch hemorrhages (orange-pink-colored intraretinal hemorrhage), angioid streaks (breaks in Bruch's membrane radiating from the optic nerve), venous occlusion, artery occlusion, peripheral neovascularization (seafan retinopathy) with subsequent vitreous hemorrhage and tractional retinal detachment. PATHOPHYSIOLOGY Hemoglobinopathies are among the more commonly inherited diseases in humans. Hemoglobinopathies result when there is altered structure, function or production of hemoglobin. Hemoglobin is the principle protein of the erythrocyte, responsible for binding and facilitating oxygen transmission to tissues. In the four variations of sickle cell disease, systemic and ocular tissues become deprived of oxygen and undergo pathologic changes.

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