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Sickle-cell disease
Classification and external resources

Normal and sickle-shaped red blood cells
ICD-10 D57.
ICD-9 282.6
OMIM 603903
DiseasesDB 12069
MedlinePlus 000527
eMedicine med/2126 oph/490 ped/2096 emerg/26 emerg/406
MeSH C15.378.071.141.150.150
.Sickle-cell disease, or sickle-cell anaemia (SCA, also called SSA, the name of the responsible mutation) or drepanocytosis, is a genetic life-long blood disorder characterized by red blood cells that assume an abnormal, rigid, sickle shape.^ Is there a cure for sickle cell disease ?
  • sicklecell.forum - Sickle Cell Disease Forum Facts, articles, and general information about Sickle Cell Disease : Sub 10 January 2010 1:26 UTC sicklecell.forum.googlepages.com [Source type: Academic]

^ Other Sickle Cell Disorders – A description of some of the Sickle Cell disorders other than Sickle Cell Anaemia .
  • The Sickle Cell Society: Learning About Sickle Cell 19 September 2009 5:14 UTC www.sicklecellsociety.org [Source type: Academic]

^ Sickle cell disease In persons with sickle cell disease, the red blood cells become crescent or sickle-shaped and also become inflexible.

.Sickling decreases the cells' flexibility and results in a risk of various complications.^ Prevent complications of sickle cell disease.
  • Blood transfusions for sickle cell disease - Yahoo! Health 15 September 2009 2:51 UTC health.yahoo.com [Source type: Academic]
  • Health: Blood transfusions for sickle cell disease; blood type ) to make sure ... 10 January 2010 1:26 UTC www.maimonidesmed.org [Source type: Academic]
  • CIGNA - Blood transfusions for sickle cell disease 10 January 2010 1:26 UTC www.cigna.com [Source type: Academic]

^ The variant genes are call sickle cell genes.
  • Sickle Cell Anemia Symptoms 19 September 2009 5:14 UTC www.iifar.org [Source type: Academic]

^ Other complications of sickle-cell disease .
  • Sickle-cell Anemia - Keyword, What Are the Sickle-Cell Trait and Sickle-Cell Disease?, What Are the Effects of Sickle-Cell Disease?, Crises, Anemia 19 September 2009 5:14 UTC www.humanillnesses.com [Source type: FILTERED WITH BAYES]

.The sickling occurs because of a mutation in the haemoglobin gene.^ The sickling occurs because of a mutation in the hemoglobin gene.
  • Sickle Cell Anemia Information 15 September 2009 2:51 UTC sicklecellanemia.researchtoday.net [Source type: Academic]
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Sickle cell trait occurred as a natural mutation of the hemoglobin gene.
  • SICKLE CELL - A PARENTS GUIDE 6 February 2010 13:21 UTC www.squidoo.com [Source type: General]

^ Sickling of cells occur because of mutation in the gene.
  • Clinnovo Diseases sickle-cell-anemia - introduction 6 February 2010 13:21 UTC www.clinnovo.com [Source type: Academic]

.Life expectancy is shortened, with studies reporting an average life expectancy of 42 and 48 years for males and females, respectively.^ Life expectancy is shortened, with older studies reporting an average life expectancy of 42 and 48 years for males and females, respectively.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ Life expectancy is shortened, with studies reporting an average life expectancy of 42 and 48 years for males and females, respectively.
  • Sickle Cell Anemia Information 15 September 2009 2:51 UTC sicklecellanemia.researchtoday.net [Source type: Academic]
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Life expectancy among patients is substantially shortened.
  • Sickle cell anaemia grips 18 percent of Chhattisgarh’s people 15 September 2009 2:51 UTC www.thaindian.com [Source type: News]

[1]
.Sickle-cell disease, usually presenting in childhood, occurs more commonly in people (or their descendants) from parts of tropical and sub-tropical regions where malaria is or was common.^ Is there a cure for sickle cell disease ?
  • sicklecell.forum - Sickle Cell Disease Forum Facts, articles, and general information about Sickle Cell Disease : Sub 10 January 2010 1:26 UTC sicklecell.forum.googlepages.com [Source type: Academic]

^ The following are common symptoms of sickle cell disease.
  • Sickle cell anaemia - information, symptoms and treatments 19 September 2009 5:14 UTC hcd2.bupa.co.uk [Source type: FILTERED WITH BAYES]

^ Malaria and sickle cells?
  • WikiAnswers - What is the connection between sickle cell anemia and malaria 6 February 2010 13:21 UTC wiki.answers.com [Source type: FILTERED WITH BAYES]

.One-third of all indigenous inhabitants of Sub-Saharan Africa carry the gene[2], because in areas where malaria is common, there is a survival value in carrying only a single sickle-cell gene (sickle cell trait).^ Sickle cell trait is different.
  • Sickle Cell Information Center News Page 19 September 2009 5:14 UTC www.scinfo.org [Source type: Academic]

^ Is there a cure for sickle cell disease ?
  • sicklecell.forum - Sickle Cell Disease Forum Facts, articles, and general information about Sickle Cell Disease : Sub 10 January 2010 1:26 UTC sicklecell.forum.googlepages.com [Source type: Academic]

^ In areas where malaria was a problem, children who inherited one sickle gene - and who, therefore, carried the sickle cell trait - survived the malaria epidemics, unlike the children who had normal hemoglobin genes.
  • Sickle Cell Disease Community Advocates - Sickle Cell Facts 10 January 2010 1:26 UTC www.scdca.org [Source type: FILTERED WITH BAYES]

[3] .Those with only one of the two alleles of the sickle-cell disease are more resistant to malaria, since the infestation of the malaria plasmodium is halted by the sickling of the cells which it infests.^ Is there a cure for sickle cell disease ?
  • sicklecell.forum - Sickle Cell Disease Forum Facts, articles, and general information about Sickle Cell Disease : Sub 10 January 2010 1:26 UTC sicklecell.forum.googlepages.com [Source type: Academic]

^ Malaria and sickle cells?
  • WikiAnswers - What is the connection between sickle cell anemia and malaria 6 February 2010 13:21 UTC wiki.answers.com [Source type: FILTERED WITH BAYES]

^ Sickle cell disease and associated hemoglobinopathies.
  • Blood transfusions for sickle cell disease - Yahoo! Health 15 September 2009 2:51 UTC health.yahoo.com [Source type: Academic]
  • Sickle Cell Disease: Topic Overview: NorthShore University HealthSystem 10 January 2010 1:26 UTC www.northshore.org [Source type: Academic]
  • CIGNA - Blood transfusions for sickle cell disease 10 January 2010 1:26 UTC www.cigna.com [Source type: Academic]
  • Bone marrow transplant for sickle cell disease - Providence Health & Services 10 January 2010 1:26 UTC www.providence.org [Source type: Academic]
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

.The prevalence of the disease in the United States is approximately 1 in 5,000, mostly affecting Americans of Sub-Saharan African descent, according to the National Institutes of Health.^ In the United States, the disease affects about 70,000 people-mainly African Americans.
  • Sickle Cell Anemia, Key Points 15 September 2009 2:51 UTC www.nhlbi.nih.gov [Source type: Academic]

^ Approximately 80,000 Americans have this disease.
  • MODEP: Minority Organ Donation Education Program, Inc. 10 January 2010 1:26 UTC modep.buffalo.edu [Source type: Academic]

^ In the United States, sickle cell disease most commonly affects African-Americans.
  • Mission Health - Genetics, Sickle Cell Disease - Asheville, Western NC 10 January 2010 1:26 UTC www.missionhospitals.org [Source type: Academic]

[4] .In the United States, about 1 in 500 black births have sickle-cell anemia.^ Symptoms of sickle cell anemia include: .

^ What is Sickle Cell Anemia shows cell shapes .
  • Sickle Cell Anemia 19 September 2009 5:14 UTC www.uni.edu [Source type: Academic]

^ Product ratings for sickle cell anemia .
  • Sickle Cell Anemia 10 January 2010 1:26 UTC www.publix.com [Source type: Academic]
  • Sickle Cell Anemia 10 January 2010 1:26 UTC www.nutritionfocus.org [Source type: Academic]
  • Health Library - 10 January 2010 1:26 UTC myhealth.ucsd.edu [Source type: Academic]
  • Trinity, Texas - Sickle Cell Anemia; Sickle cell anemia may result in vitamin ... 6 February 2010 13:21 UTC www.tmfhs.org [Source type: Academic]
  • Sickle Cell Anemia 6 February 2010 13:21 UTC www.naturalmedicine.com [Source type: Academic]
  • Citizens Health Information 6 February 2010 13:21 UTC www.citizensmemorial.com [Source type: Academic]
  • Sickle Cell Anemia | Natural Path 6 February 2010 13:21 UTC www.naturalpath.com [Source type: Academic]
  • Join Truestar now and achieve total health and weight loss now! 6 February 2010 13:21 UTC www.truestarhealth.com [Source type: Academic]
  • Delicious Living - for the organic, green and healthy lifestyle 6 February 2010 13:21 UTC healthnotes.deliciouslivingmag.com [Source type: Academic]

Contents

Pathophysiology

.Normally, we have Haemoglobin A (consists of 2 Alpha and 2 Beta chains), Haemoglobin A2 (consists of 2 Alpha and 2 Delta chains) and Haemoglobin F (consists of 2 Alpha and 2 Gamma Chains) in our bodies.Haemoglobin A makes around 96-97% of the normal haemoglobin in humans.^ Gene for normal hemoglobin beta chain .
  • The Molecular Biology of Sickle Cell Anemia 15 September 2009 2:51 UTC www.nslc.wustl.edu [Source type: Academic]

^ Haemoglobin H is produced by four normal b chains.

^ It is made up of alpha chains and beta chains.
  • Sickle Cell Disease 15 September 2009 2:51 UTC kidshealth.org [Source type: Academic]
  • KidsHealth - Sickle Cell Disease - University of Minnesota Children''s Hospital, Fairview 10 January 2010 1:26 UTC www.uofmchildrenshospital.org [Source type: Academic]
  • Sickle Cell Disease 2009-07-01 00:00:00.0 10 January 2010 1:26 UTC www.devoschildrens.org [Source type: Academic]
  • KidsHealth - Sickle Cell Disease - University of Minnesota Children''s Hospital, Fairview 10 January 2010 1:26 UTC www.uofmchildrenshospital.org [Source type: Academic]

.In normal Haemoglobin A, glutamic acid is on the 6th position of the beta chain, while in sickle-cell disease, this glutamic acid is replaced by valine leading to the formation of sickle cells.^ The sickle cell anemia mutation is in the beta chain of hemoglobin.
  • TUTORIAL: Sickle-cell Anemia 19 September 2009 5:14 UTC bioquest.org [Source type: FILTERED WITH BAYES]

^ Sickle cell disease and associated hemoglobinopathies.
  • Blood transfusions for sickle cell disease - Yahoo! Health 15 September 2009 2:51 UTC health.yahoo.com [Source type: Academic]
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Sickle Cell Disease: Topic Overview: NorthShore University HealthSystem 10 January 2010 1:26 UTC www.northshore.org [Source type: Academic]
  • CIGNA - Blood transfusions for sickle cell disease 10 January 2010 1:26 UTC www.cigna.com [Source type: Academic]
  • Bone marrow transplant for sickle cell disease - Providence Health & Services 10 January 2010 1:26 UTC www.providence.org [Source type: Academic]
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Pulmonary hypertension in sickle cell disease.
  • Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia -- Gladwin and Kato 2005 (1): 51 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

.This happens due to a one point mutation.^ It is an autosomal recessive disorder caused due to a point mutation in hemoglobin beta gene on chromosome 11p15.4.
  • Sleeping Beauty Transposon System Shows Promise In Sickle Cell Anemia | MediNEWS.Direct! 6 February 2010 13:21 UTC www.medinewsdirect.com [Source type: Academic]

^ Are there any evidence that > > > > point to a weaker race due to genetic mutations and drift?
  • How does Religion account for Race? - talk.origins | Google Groups 10 January 2010 1:26 UTC groups.google.com [Source type: FILTERED WITH BAYES]

^ The neurological complications result from one point mutation that causes vasculopathy of both large and small vessels.

.This leads to polymerization of the two beta chains and therefore their appearance as puzzle pieces (or lock and key); which means they fit into each other forming a longitudinal polymer that would lead to the cell becoming deformed and very rigid leading to vessel occlusion.^ The cells become abnormally rigid and sickle shaped, meaning they can get trapped in small blood vessels.
  • Sickle cell disease: striving for better treatment | Action Medical Research 10 January 2010 1:26 UTC www.action.org.uk [Source type: Academic]

^ This may prevent the cells from becoming rigid and clogging the blood vessels.
  • Clinical Alert: Drug Treatment for Sickle Cell Anemia 19 September 2009 5:14 UTC www.nlm.nih.gov [Source type: Academic]

^ Occlusions in blood vessels can lead to a painful sickle cell crisis.
  • Pain Medicine & Palliative Care 10 January 2010 1:26 UTC www.stoppain.org [Source type: Academic]

.This process of polymerization can be activated by infections, hypoxia, acidosis, physical exercise, vasoocclusion due to cold as well as hypertonic dehydration.^ The crisis provoked by infection, cold or dehydration.
  • Difference between sickle cell trait and sickle cell anemia. Anemia is defined as a condition in which the concentration 6 February 2010 13:21 UTC ndri.com [Source type: Academic]

^ The pathophysiology of the disease is complex, but is ultimately due to the beta-globin point mutation that allows HbS polymerization to occur under conditions of hypoxia, acidemia, fever, and erythrocyte dehydration.
  • Modulation of Fetal Hemoglobin in Sickle Cell Anemia 6 February 2010 13:21 UTC cme.medscape.com [Source type: Academic]

^ Dehydration Infection Fever Hypoxia (decrease in oxygen to body tissue) Bleeding Cold exposure Drug and alcohol use Pregnancy and stress Fourpatterns of an acute sickle cell crisis are now recognizable.

Classification

.Sickle-cell anaemia is the name of a specific form of sickle-cell disease in which there is homozygosity for the mutation that causes HbS. Sickle-cell anaemia is also referred to as "HbSS", "SS disease", "haemoglobin S" or permutations thereof.^ Is there a cure for sickle cell disease ?
  • sicklecell.forum - Sickle Cell Disease Forum Facts, articles, and general information about Sickle Cell Disease : Sub 10 January 2010 1:26 UTC sicklecell.forum.googlepages.com [Source type: Academic]

^ What kinds of problems does Sickle Cell Disease cause?
  • Sickle Cell Donation | Sickle Cell Donation 19 September 2009 5:14 UTC www.pleasegiveblood.org [Source type: FILTERED WITH BAYES]

^ There is no cure for sickle cell.
  • Living with Sickle Cell Anemia | Firstcoastnews.com | Local News 6 February 2010 13:21 UTC www.firstcoastnews.com [Source type: General]
  • Living with Sickle Cell Anemia | Firstcoastnews.com | News 6 February 2010 13:21 UTC www.firstcoastnews.com [Source type: General]

.In heterozygous people, who have only one sickle gene and one normal adult haemoglobin gene, it is referred to as "HbAS" or "sickle cell trait". Other, rarer forms of sickle-cell disease include sickle-haemoglobin C disease (HbSC), sickle beta-plus-thalassaemia (HbS/β+) and sickle beta-zero-thalassaemia (HbS/β0).^ Sickle cell trait is different.
  • Sickle Cell Information Center News Page 19 September 2009 5:14 UTC www.scinfo.org [Source type: Academic]

^ Who should be tested for sickle cell disease?

^ Symptoms of sickle cell anemia include: .
  • Sickle Cell Anemia - Sickle Cell Anemia Description - Sickle Cell Anemia Symptoms - Sickle Cell Anemia Prevention - Sickle Cell Anemia Treatment 6 February 2010 13:21 UTC symptomchecker.about.com [Source type: FILTERED WITH BAYES]
  • Pri-Med Patient Education Center - Sickle Cell Anemia 6 February 2010 13:21 UTC www.patienteducationcenter.org [Source type: FILTERED WITH BAYES]

.These other forms of sickle-cell disease are compound heterozygous states in which the person has only one copy of the mutation that causes HbS and one copy of another abnormal haemoglobin allele.^ Stroke – another sudden and severe complication of persons with sickle cell disease.
  • VCU Massey Cancer Center :: Sickle cell diseases - Signs and symptoms 10 January 2010 1:26 UTC www.massey.vcu.edu [Source type: Academic]

^ Is there a cure for sickle cell disease ?
  • sicklecell.forum - Sickle Cell Disease Forum Facts, articles, and general information about Sickle Cell Disease : Sub 10 January 2010 1:26 UTC sicklecell.forum.googlepages.com [Source type: Academic]

^ It occurs when a person inherits the usual haemoglobin from oneparent and sickle haemoglobin from the other.
  • Union of Shop, Distributive and Allied Workers: Resources Library: Sickle Cell Anaemia 15 September 2009 2:51 UTC www.usdaw.org.uk [Source type: FILTERED WITH BAYES]

.The term disease is applied, because the inherited abnormality causes a pathological condition that can lead to death and severe complications.^ Infections and lung disease are leading causes of death.
  • Hydroxyurea Treatment for Sickle Cell Disease 19 September 2009 5:14 UTC consensus.nih.gov [Source type: Academic]

^ Infections are the leading cause of death in sickle cell disease.
  • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 10 January 2010 1:26 UTC www.chop.edu [Source type: Academic]
  • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 6 February 2010 13:21 UTC www.chop.edu [Source type: Academic]

^ It is an often unrecognized complication and cause of death in sickle cell disease.
  • Sickle cell disease: the causes, diagnosis, and treatment of sickle ... 10 January 2010 1:26 UTC www.henryfordhealth.org [Source type: Academic]
  • Sickle cell disease - Complications 10 January 2010 1:26 UTC www.umm.edu [Source type: Academic]
  • Sickle cell disease 10 January 2010 1:26 UTC www.fitsugar.com [Source type: Academic]
  • Sickle Cell Anemia Complications - Sickle Cell Anemia Health Information - NY Times Health 6 February 2010 13:21 UTC health.nytimes.com [Source type: Academic]

.Not all inherited variants of haemoglobin are detrimental, a concept known as genetic polymorphism.^ Not all inherited variants of haemoglobin are detrimental, a concept known as genetic polymorphism.

^ Not all inherited variants of haemoglobin are detrimental, a concept known as genetic polymorphism .
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ The reason for this, as evolutionist Theodosius Dobzhansky points out, is that "the process of mutation is the only known source of the new materials of genetic variability, and hence evolution."
  • Sickle Cell Anemia and Other Good Mutations of Evolution by David Menton 6 February 2010 13:21 UTC www.gennet.org [Source type: FILTERED WITH BAYES]

Signs and symptoms

.Sickle-cell disease may lead to various acute and chronic complications, several of which are potentially lethal.^ Is there a cure for sickle cell disease ?
  • sicklecell.forum - Sickle Cell Disease Forum Facts, articles, and general information about Sickle Cell Disease : Sub 10 January 2010 1:26 UTC sicklecell.forum.googlepages.com [Source type: Academic]

^ The severity of sickle cell disease generally depends on a number of factors: .
  • Sickle cell disease: the causes, diagnosis, and treatment of sickle ... 10 January 2010 1:26 UTC www.henryfordhealth.org [Source type: Academic]
  • Sickle cell disease - Edward Hospital, Naperville IL Area 10 January 2010 1:26 UTC www.edward.org [Source type: Academic]
  • HowStuffWorks "Sickle Cell Disease In-Depth" 10 January 2010 1:26 UTC healthguide.howstuffworks.com [Source type: Academic]
  • Sickle cell disease 10 January 2010 1:26 UTC www.bcbswny.com [Source type: Academic]
  • Sickle cell disease 10 January 2010 1:26 UTC www.fitsugar.com [Source type: Academic]

^ Sickle cell disease and associated hemoglobinopathies.
  • Blood transfusions for sickle cell disease - Yahoo! Health 15 September 2009 2:51 UTC health.yahoo.com [Source type: Academic]
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Sickle Cell Disease: Topic Overview: NorthShore University HealthSystem 10 January 2010 1:26 UTC www.northshore.org [Source type: Academic]
  • CIGNA - Blood transfusions for sickle cell disease 10 January 2010 1:26 UTC www.cigna.com [Source type: Academic]
  • Bone marrow transplant for sickle cell disease - Providence Health & Services 10 January 2010 1:26 UTC www.providence.org [Source type: Academic]

Crisis

.Sickle cell disease results in anemia and crisis that could be of many types including The vaso-occlusive crisis , Aplastic Crisis, Sequestration Crisis, Hyper hemolytic crisis and others.^ Symptoms of sickle cell anemia include: .

^ Is there a cure for sickle cell disease ?
  • sicklecell.forum - Sickle Cell Disease Forum Facts, articles, and general information about Sickle Cell Disease : Sub 10 January 2010 1:26 UTC sicklecell.forum.googlepages.com [Source type: Academic]

^ Sickle cell anemia is also known as Hemoglobin S disease.

.
  • 'The vaso-occlusive crisis: which is caused by sickle-shaped red blood cells that obstruct capillaries and restrict blood flow to an organ, resulting in ischemia (ischaemia), pain, necrosis and often organ damage.^ Sickle cell crisis splenectomy increased haemoglobin and red blood cell survival.
    • Evidence-Based On-Call 15 September 2009 2:51 UTC www.eboncall.org [Source type: Academic]

    ^ What is Sickle Cell Anemia shows cell shapes .
    • Sickle Cell Anemia 19 September 2009 5:14 UTC www.uni.edu [Source type: Academic]

    ^ Sickle cells contain abnormal hemoglobin that causes the cells to have a sickle shape.
    • Sickle Cell Anemia, What Is 15 September 2009 2:51 UTC www.nhlbi.nih.gov [Source type: FILTERED WITH BAYES]
    • Sickle Cell Anemia, All Sections 15 September 2009 2:51 UTC www.nhlbi.nih.gov [Source type: FILTERED WITH BAYES]
    • healthyNJ--Information for Healthy Living--Sickle Cell Anemia 6 February 2010 13:21 UTC www.healthynj.org [Source type: Academic]
    • Sickle Cell Anemia News, Research 6 February 2010 13:21 UTC www.news-medical.net [Source type: Academic]

    .The frequency, severity, and duration of these crises vary considerably.^ These severe attacks are known as Crises .

    ^ The frequency and duration of the pain can vary tremendously.
    • Sickle-cell anemia, Information about Sickle-cell anemia 19 September 2009 5:14 UTC www.faqs.org [Source type: Academic]

    ^ The frequency, severity, and duration of these crises varies considerably.
    • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

    .Painful crises are treated with hydration and analgesics; pain management requires opioid administration at regular intervals until the crisis has settled.^ Moderate-to-severe pain is treated with opioids.
    • Sickle Cell Anemia Symptoms 19 September 2009 5:14 UTC www.iifar.org [Source type: Academic]

    ^ Painful crises are treated with hydration and analgesics; pain management requires opioid administration at regular intervals until the crisis has settled.

    ^ Many pain crises can be managed at home.
    • Your Child: University of Michigan Health System 10 January 2010 1:26 UTC www.med.umich.edu [Source type: FILTERED WITH BAYES]

    .For milder crises, a subgroup of patients manage on NSAIDs (such as diclofenac or naproxen).^ For milder crises a subgroup of patients manage on NSAIDs (such as diclofenac or naproxen ).
    • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

    ^ For milder crises, a subgroup of patients manage on NSAIDs (such as diclofenac or naproxen).

    ^ Some patients with severe symptoms receive regular blood transfusions to prevent crises and/or other complications such as stroke and organ damage.
    • Sickle Cell Anemia - baby, symptoms, meaning, Definition, Description, Demographics, Causes and symptoms, Diagnosis, Treatment, Prognosis, Prevention 6 February 2010 13:21 UTC www.healthofchildren.com [Source type: Academic]

    .For more severe crises, most patients require inpatient management for intravenous opioids; patient-controlled analgesia (PCA) devices are commonly used in this setting.^ Older patients with severe pain may also require intravenous administration.
    • Sickle cell disease - Edward Hospital, Naperville IL Area 10 January 2010 1:26 UTC www.edward.org [Source type: Academic]
    • Sickle cell disease 10 January 2010 1:26 UTC www.bcbswny.com [Source type: Academic]

    ^ For more severe crises most patients require inpatient management for intravenous opioids; patient-controlled analgesia (PCA) devices are commonly used in this setting.
    • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

    ^ For more severe crises, most patients require inpatient management for intravenous opioids; patient-controlled analgesia (PCA) devices are commonly used in this setting.

    .Diphenhydramine is sometimes effective for the itching associated with the opioid use.^ Diphenhydramine is sometimes effective for the itching associated with the opioid use.

    ^ Diphenhydramine is effective for the itching associated with the opioid use.
    • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

    ^ Diphenhydramine is also an effective agent that is frequently prescribed by doctors in order to help control any itching associated with the use of opioids.

    Incentive spirometry, a technique to encourage deep breathing to minimise the development of atelectasis, is recommended.
.Because of its narrow vessels and function in clearing defective red blood cells, the spleen is frequently affected.^ Important in red blood cell production.
  • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]
  • MoonDragon's ObGyn Information: Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: FILTERED WITH BAYES]

^ Irregular red blood cell shape .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Sickle cell refers to the sickle (crescent) shape of affected red blood cells.
  • U.S. Army HOOAH 4 HEALTH - HOOAH 4 FAMILY - Sickle Cell Anemia 6 February 2010 13:21 UTC www.hooah4health.com [Source type: Academic]

.It is usually infarcted before the end of childhood in individuals suffering from sickle-cell anaemia.^ "Sickle cell anaemia trait".
  • Sickle Cell Patients Resource - www.sicklecell.md 10 January 2010 1:26 UTC www.sicklecell.md [Source type: Academic]

^ "Sickle cell anaemia disease".
  • Sickle Cell Patients Resource - www.sicklecell.md 10 January 2010 1:26 UTC www.sicklecell.md [Source type: Academic]

^ People with sickle cell anaemia do suffer from malaria, and very badly too.
  • Sickle Cell Patients Resource - www.sicklecell.md 10 January 2010 1:26 UTC www.sicklecell.md [Source type: Academic]

.This autosplenectomy increases the risk of infection from encapsulated organisms;[5][6] preventive antibiotics and vaccinations are recommended for those with such asplenia.^ Antibiotics may be given to prevent infections.
  • Sickle-cell Anemia - Keyword, What Are the Sickle-Cell Trait and Sickle-Cell Disease?, What Are the Effects of Sickle-Cell Disease?, Crises, Anemia 19 September 2009 5:14 UTC www.humanillnesses.com [Source type: FILTERED WITH BAYES]

^ Antibiotics – medication given to prevent infections .
  • Caregivers: Sickle Cell Disease - NetofCare 10 January 2010 1:26 UTC www.netofcare.org [Source type: Academic]
  • Pain Medicine & Palliative Care 19 September 2009 5:14 UTC www.wehealny.org [Source type: Academic]

^ Antibiotics such as penicillin help prevent dangerous infections.
  • NJDHSS - Sickle Cell Disease 10 January 2010 1:26 UTC www.state.nj.us [Source type: FILTERED WITH BAYES]

.One of the earliest clinical manifestations is dactylitis, presenting as early as six months of age, and may occur in children with sickle trait.^ Persons with sickle cell trait may pass the sickle gene to their children.
  • Sickle Cell Anemia 6 February 2010 13:21 UTC dwb.unl.edu [Source type: FILTERED WITH BAYES]

^ Overwhelming infection may be the presenting manifestation of sickle cell anemia in early childhood.
  • Sickle Cell Anemia - New Treatments, August 1, 2009 6 February 2010 13:21 UTC www.ccspublishing.com [Source type: Academic]

^ Dactylitis is a common early manifestation that may occur before the age of 6 months.
  • Sickle Cell Disease and Sickle Cell Anaemia | Doctor | Patient UK 10 January 2010 1:26 UTC www.patient.co.uk [Source type: FILTERED WITH BAYES]

[7] .The crisis can last up to a month.^ The crisis can last up to a month.

[8] .Another recognised type of sickle crisis is the acute chest syndrome, a condition characterised by fever, chest pain, difficulty breathing, and pulmonary infiltrate on a chest X-ray.^ Effect of transfusion in acute chest syndrome of sickle cell disease.
  • The acute chest syndrome in children and adolescents with sickle cell disease 10 January 2010 1:26 UTC www.uptodate.com [Source type: Academic]

^ The acute chest syndrome in children and adolescents with sickle cell disease .
  • The acute chest syndrome in children and adolescents with sickle cell disease 10 January 2010 1:26 UTC www.uptodate.com [Source type: Academic]

^ Acute chest syndrome is a particularly serious complication of sickle-cell crisis.

.Given that pneumonia and sickling in the lung can both produce these symptoms, the patient is treated for both conditions.^ This condition produces a group of symptoms known as a sickle cell crisis.
  • Sickle Cell Anemia - HealthLibrary 10 January 2010 1:26 UTC healthlibrary.epnet.com [Source type: Academic]
  • Sickle Cell Anemia – UPMC, Pittsburgh, PA, USA 6 February 2010 13:21 UTC www.upmc.com [Source type: Academic]
  • Health Encyclopedia | Butler Hospital Providence, RI 6 February 2010 13:21 UTC www.butler.org [Source type: FILTERED WITH BAYES]
  • Health NJ - Sickle Cell Anemia - Somerset Medical, Somerville, Central NJ 6 February 2010 13:21 UTC www.somersetmedicalcenter.com [Source type: Academic]
  • Tufts Medical Center - Sickle Cell Anemia 6 February 2010 13:21 UTC www.tufts-nemc.org [Source type: Academic]
  • Conditions In Brief - The Doctors Clinic 6 February 2010 13:21 UTC www.thedoctorsclinic.com [Source type: Academic]
  • Sickle Cell Anemia - Content Viewer 6 February 2010 13:21 UTC www.svmh.com [Source type: Academic]
  • Northern Arizona Healthcare - Sickle Cell Anemia 6 February 2010 13:21 UTC www.nahealth.com [Source type: Academic]
  • Health Information Details 6 February 2010 13:21 UTC www.ridgeviewmedical.org [Source type: Academic]

^ These can be secondary to either an acute lung infection (pneumonia) or sickling in the lung.
  • NJDHSS - Sickle Cell Disease 10 January 2010 1:26 UTC www.nj.gov [Source type: Academic]

^ Given that pneumonia and sickling in the lung can both produce these symptoms, the patient is treated for both conditions.

.July 2008" style="white-space:nowrap;">[citation needed] It can be triggered by painful crisis, respiratory infection, bone-marrow embolisation, or possibly by atelectasis, opiate administration, or surgery.^ Vasoocclusive crisis is often triggered by infection.
  • Sickle Cell Anemia: eMedicine Hematology 6 February 2010 13:21 UTC emedicine.medscape.com [Source type: Academic]

^ Painful events are commonly triggered by infection.
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Evansville,IN Sickle Cell Disease; Sickle cell disease changes normal, ... 10 January 2010 1:26 UTC www.stmarys.org [Source type: Academic]

^ It can be triggered by painful crisis, respiratory infection, bone-marrow embolisation, or possibly by atelectasis, opiate administration, or surgery.

.Most episodes of sickle cell crises last between five and seven days.^ Most individuals with sickle cell disease are generally well from day-to-day.
  • Center for Cancer and Blood Disorders > Sickle Cell FAQ 10 January 2010 1:26 UTC www.childrens.com [Source type: Academic]

^ Almost all patients with sickle cell anemia have painful episodes (crises), which can last from hours to days.
  • Sickle cell anemia: MedlinePlus Medical Encyclopedia 10 January 2010 1:26 UTC www.nlm.nih.gov [Source type: Academic]
  • Sickle cell anemia - Health Diseases & Medical Conditions - South Florida Hospital 10 January 2010 1:26 UTC www.mhs.net [Source type: Academic]
  • Sickle Cell Anemia - Symptoms, Diagnosis, Treatment of Sickle Cell Anemia - NY Times Health Information 10 January 2010 1:26 UTC health.nytimes.com [Source type: Academic]
  • DC Health - Sickle cell anemia. Sickle cell anemia is a disease passed down... 10 January 2010 1:26 UTC www.whcenter.org [Source type: Academic]
  • Sickle cell anemia - Shands Healthcare 10 January 2010 1:26 UTC shands.org [Source type: Academic]
  • Sickle cell anemia - Scripps Health - San Diego 6 February 2010 13:21 UTC mobile.scripps.org [Source type: Academic]
  • DHMC | Health Encyclopedia | Sickle cell anemia 6 February 2010 13:21 UTC www.dhmc.org [Source type: Academic]
  • BHS, Pittsfield - Sickle cell anemia. Sickle cell anemia is a disease passed... 6 February 2010 13:21 UTC www.berkshirehealthsystems.com [Source type: Academic]
  • Sickle cell anemia Medical Information 6 February 2010 13:21 UTC www.drugs.com [Source type: Academic]
  • Sickle cell anemia 6 February 2010 13:21 UTC symptomnav.adam.com [Source type: Academic]
  • Sickle cell anemia- medco.com 6 February 2010 13:21 UTC www.medcohealth.com [Source type: Academic]
  • Sickle cell anemia - Adventist HealthCare 6 February 2010 13:21 UTC www.adventisthealthcare.com [Source type: Academic]
  • Sickle cell anemia - Hillcrest Health System 6 February 2010 13:21 UTC hillcrest2.adam.com [Source type: Academic]
  • Sickle cell anemia 6 February 2010 13:21 UTC ssmhealth.adam.com [Source type: Academic]
  • Sickle cell anemia 6 February 2010 13:21 UTC www.pennmedicine.org [Source type: Academic]

^ Pain episodes are the most common symptom of sickle cell disease (2).
  • Charles Curtis-Thomas, RN, MD 10 January 2010 1:26 UTC www.cwru.edu [Source type: Academic]

[9]
.
  • Splenic sequestration crises: are acute, painful enlargements of the spleen.The sinusoids and gates would open at the same time resulting in sudden pooling of the blood into the spleen and circulatory defect leading to sudden hypovolemia The abdomen becomes bloated and very hard.Splenic sequestration crises is considered an emergency.If not treated, patients may die within 1-2 hours due to circulatory failure.Management is supportive, sometimes with blood transfusion.This crises is transient, it continues for 3-4 hours and may last for one day.
  • Aplastic crises are acute worsenings of the patient's baseline anaemia, producing pallor, tachycardia, and fatigue.^ Splenic sequestration (pooling) crises happen when sickle cells pool in the spleen.
    • Departments & Programs - Children's National Medical Center 10 January 2010 1:26 UTC www.childrensnational.org [Source type: Academic]

    ^ Sometimes pain lasts only a few hours; sometimes it lasts several weeks.
    • Sickle Cell Anemia Disease Profile 10 January 2010 1:26 UTC www.ornl.gov [Source type: Academic]

    ^ The anemia may become so severe that a person will need to have a blood transfusion.
    • Sickle-cell Anemia - Keyword, What Are the Sickle-Cell Trait and Sickle-Cell Disease?, What Are the Effects of Sickle-Cell Disease?, Crises, Anemia 19 September 2009 5:14 UTC www.humanillnesses.com [Source type: FILTERED WITH BAYES]

    .This crisis is triggered by parvovirus B19, which directly affects erythropoiesis (production of red blood cells) by invading the red cell precursors and multiplying in them and destroying them.^ Important in red blood cell production.
    • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]

    ^ The disease affects the red blood cells.
    • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 10 January 2010 1:26 UTC www.chop.edu [Source type: Academic]
    • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 6 February 2010 13:21 UTC www.chop.edu [Source type: Academic]

    ^ Parvovirus infection nearly completely prevents red blood cell production for 2-3 days.
    • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

    .Parvovirus infection nearly completely prevents red blood cell production for two to three days.^ Important in red blood cell production.
    • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]
    • MoonDragon's ObGyn Information: Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: FILTERED WITH BAYES]

    ^ Irregular red blood cell shape .
    • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

    ^ The disease affects the red blood cells.
    • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 10 January 2010 1:26 UTC www.chop.edu [Source type: Academic]
    • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 6 February 2010 13:21 UTC www.chop.edu [Source type: Academic]

    .In normal individuals, this is of little consequence, but the shortened red cell life of sickle-cell patients results in an abrupt, life-threatening situation.^ Normal red blood cells are round.
    • Sickle Cell Anemia 6 February 2010 13:21 UTC my.clevelandclinic.org [Source type: Academic]
    • MayoClinic.com Health Library - Sickle cell anemia 6 February 2010 13:21 UTC www.riversideonline.com [Source type: FILTERED WITH BAYES]

    ^ This is a result of damage to the spleen from the sickled red cells.
    • Center for Cancer and Blood Disorders > Sickle Cell FAQ 10 January 2010 1:26 UTC www.childrens.com [Source type: Academic]

    ^ Normal red blood cell .

    .Reticulocyte counts drop dramatically during the disease, and the rapid turnover of red cells leads to the drop in haemoglobin.^ The disease affects the red blood cells.
    • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 10 January 2010 1:26 UTC www.chop.edu [Source type: Academic]
    • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 6 February 2010 13:21 UTC www.chop.edu [Source type: Academic]

    ^ RETICULOCYTE OR RETIC COUNT Reticulocytes are young red blood cells.
    • NJDHSS - Sickle Cell Disease 10 January 2010 1:26 UTC www.newjersey.gov [Source type: FILTERED WITH BAYES]

    ^ This can lead to a serious and rapid drop in the red cell count (severe anemia).
    • Sickle Cell Disease 15 September 2009 2:51 UTC kidshealth.org [Source type: Academic]
    • KidsHealth - Sickle Cell Disease - University of Minnesota Children''s Hospital, Fairview 10 January 2010 1:26 UTC www.uofmchildrenshospital.org [Source type: Academic]
    • Sickle Cell Disease 2009-07-01 00:00:00.0 10 January 2010 1:26 UTC www.devoschildrens.org [Source type: Academic]
    • KidsHealth - Sickle Cell Disease - University of Minnesota Children''s Hospital, Fairview 10 January 2010 1:26 UTC www.uofmchildrenshospital.org [Source type: Academic]

    .This crisis takes 4 days to one week to disappear.Most patients can be managed supportively; some need blood transfusion.
  • Hemolytic crises are acute accelerated drops in haemoglobin level.^ Management is supportive, sometimes with blood transfusion .

    ^ It also reduces the need for blood transfusions.

    ^ Blood transfusions carry some risk.
    • Treatments for Sickle Cell 10 January 2010 1:26 UTC www.netwellness.org [Source type: Academic]
    • Sickle cell anemia: Treatments and drugs - MayoClinic.com 10 January 2010 1:26 UTC www.mayoclinic.com [Source type: Academic]
    • Sickle cell anemia - Sickle cell disease (SCD) 10 January 2010 1:26 UTC www.dreddyclinic.com [Source type: Academic]
    • Sickle cell anemia - Valley Health System 6 February 2010 13:21 UTC www.valleyhealth.com [Source type: FILTERED WITH BAYES]
    • Sickle cell anemia 6 February 2010 13:21 UTC www.cnn.com [Source type: FILTERED WITH BAYES]
    • MayoClinic.com Health Library - Sickle cell anemia 6 February 2010 13:21 UTC www.riversideonline.com [Source type: FILTERED WITH BAYES]
    • Sickle cell anemia 6 February 2010 13:21 UTC edition.cnn.com [Source type: FILTERED WITH BAYES]

    .The red blood cells break down at a faster rate.^ Important in red blood cell production.
    • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]
    • MoonDragon's ObGyn Information: Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: FILTERED WITH BAYES]

    ^ The disease affects the red blood cells.
    • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 10 January 2010 1:26 UTC www.chop.edu [Source type: Academic]
    • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 6 February 2010 13:21 UTC www.chop.edu [Source type: Academic]

    ^ When red blood cells sickle, they break down prematurely, which can lead to anemia.
    • Sickle cell disease - Genetics Home Reference 10 January 2010 1:26 UTC ghr.nlm.nih.gov [Source type: Academic]
    • Breakthrough For Sickle Cell Anemia 19 September 2009 5:14 UTC www.health-reports.com [Source type: FILTERED WITH BAYES]
    • Sickle cell disease - Genetics Home Reference 6 February 2010 13:21 UTC ghr.nlm.nih.gov [Source type: Academic]

    .This is particularly common in patients with co-existent G6PD deficiency.^ This is particularly common in patients with co-existent G6PD deficiency .
    • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

    ^ This is particularly common in patients with co-existent G6PD deficiency.

    ^ BACKGROUND: Iron deficiency anemia is a common complication in patients with chronic kidney disease (CKD).
    • Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC www.kosmix.com [Source type: Academic]

    Management is supportive, sometimes with blood transfusions.

Complications

Sickle-cell anaemia can lead to various complications, including:
.
  • Overwhelming post-(auto)splenectomy infection (OPSI), which is due to functional asplenia, caused by encapsulated organisms such as Streptococcus pneumoniae and Haemophilus influenzae.^ The most common organisms causing infection in children with sickle-cell disease are Streptococcus pneumoniae and Haemophilus influenza .

    ^ Vaccinations for pneumococcal pneumonia, Haemophilus influenza, hepatitis and influenza to prevent infections .

    ^ Overwhelming post-(auto)splenectomy infection (OPSI) is due to functional asplenia, caused by encapsulated organisms such as Streptococcus pneumoniae and Haemophilus influenzae .
    • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

    .Daily penicillin prophylaxis is the most commonly used treatment during childhood, with some haematologists (hematologists) continuing treatment indefinitely.^ Daily penicillin prophylaxis is the most commonly used treatment during childhood, with some haematologists continuing treatment indefinitely.

    ^ Most kids will require two daily doses of penicillin, as prescribed by their doctors, until they're at least 5 years old (and often older).
    • KidsHealth - Sickle Cell Disease - University of Minnesota Children''s Hospital, Fairview 10 January 2010 1:26 UTC www.uofmchildrenshospital.org [Source type: Academic]
    • KidsHealth - Sickle Cell Disease - University of Minnesota Children''s Hospital, Fairview 10 January 2010 1:26 UTC www.uofmchildrenshospital.org [Source type: Academic]

    ^ Health maintenance: For long-term management the following interventions have reduced mortality, particularly during childhood: Pneumococcal, Haemophilus influenzae , and meningococcal vaccines Early identification and treatment of serious bacterial infections Prophylactic antibiotics, including continuous prophylaxis with oral penicillin from age 4 mo to 6 yr Use of hydroxyurea Some Trade Names HYDREA Click for Drug Monograph and folate supplementation .
    • Sickle Cell Disease: Anemias Caused by Hemolysis: Merck Manual Professional 6 February 2010 13:21 UTC www.merck.com [Source type: Academic]

    .Patients benefit today from routine vaccination for H. influenzae, S. pneumoniae, and Neisseria meningitidis.
  • Stroke, which can result from a progressive narrowing of blood vessels, preventing oxygen from reaching the brain.^ Stroke When there is a blockage of blood vessels supplying the brain, a stroke results.
    • NJDHSS - Sickle Cell Disease 10 January 2010 1:26 UTC www.state.nj.us [Source type: FILTERED WITH BAYES]

    ^ These blockages prevent oxygenated blood from reaching the tissues resulting in pain.
    • Sickle Cell Anemia in African Americans! 6 February 2010 13:21 UTC www.aging-no-more.com [Source type: Academic]

    ^ If sickle-shaped cells block a blood vessel in the brain, a stroke can result.
    • Sickle Cell Disease - MSN Health & Fitness - Kids' Health 10 January 2010 1:26 UTC health.msn.com [Source type: Academic]
    • CPDX - Sickle Cell Anemia Screening 6 February 2010 13:21 UTC www.cpdx.com [Source type: Academic]

    .Cerebral infarction occurs in children and cerebral hemorrhage in adults.
  • Cholelithiasis (gallstones) and cholecystitis, which may result from excessive bilirubin production and precipitation due to prolonged haemolysis.
  • Jaundice, yellowing of the skin, may occur due to the inability of the liver to effectively remove bilirubin from the filtering of damaged red blood cells out of the blood supply as well as blocks in the organ's blood supply.^ Important in red blood cell production.
    • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]

    ^ It is characterised by sickle-shaped red blood cells which may block blood vessels.

    ^ The disease affects the red blood cells.
    • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 10 January 2010 1:26 UTC www.chop.edu [Source type: Academic]
    • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 6 February 2010 13:21 UTC www.chop.edu [Source type: Academic]

    [10][11]
  • .
  • Avascular necrosis (aseptic bone necrosis) of the hip and other major joints, which may occur as a result of ischemia.
  • Decreased immune reactions due to hyposplenism (malfunctioning of the spleen).
  • Priapism and infarction of the penis.
  • Osteomyelitis (bacterial bone infection), Salmonella is the second most common organism behind Staphylococcus aureus which remains first in both the general population and those with sickle cell.^ Decreased immune reactions due to hyposplenism (malfunctioning of the spleen) Priapism and infarction of the penis .
    • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

    ^ Individuals with sickle cell disease are also at increased risk for other infections such as osteomyelitis caused by Staphylococcus aureus or other organisms such as Salmonella species.
    • Sickle Cell Disease -- GeneReviews -- NCBI Bookshelf 10 January 2010 1:26 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

    ^ Such damage may result in avascular necrosis (especially of the femur) and bone deterioration.
    • ISPUB - Epidemiology, Population Health Genetics and Phenotypic Diversity of Sickle Cell Disease in India 10 January 2010 1:26 UTC www.ispub.com [Source type: Academic]

    .[12]
  • Opioid tolerance, which can occur as a normal, physiologic response to the therapeutic use of opiates.^ Opioid tolerance can occur as a normal, physiologic response to the therapeutic use of opiates.
    • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

    ^ Opioid tolerance, which can occur as a normal, physiologic response to the therapeutic use of opiates.

    ^ "But in the absence of a maternal immune response, we confirmed that immune tolerance does occur in the early-gestation fetus 100 percent of the time."
    • Sickle Cell Information Center News Page 19 September 2009 5:14 UTC www.scinfo.org [Source type: Academic]

    .Addiction to opiates occurs no more commonly among individuals with sickle-cell disease than among other individuals treated with opiates for other reasons.
  • Acute papillary necrosis in the kidneys.
  • Leg ulcers.
  • In eyes, background retinopathy, proliferative retinopathy, vitreous haemorrhages and retinal detachments, resulting in blindness.^ Bleeding can also occur within the eye (retinal hemorrhage) and retinal detachment can result.
    • Sickle Cell Anemia | Sickle Cell Disease | Trait | MyDocHub.com 6 February 2010 13:21 UTC www.mydochub.com [Source type: Academic]

    ^ Sickle cell disease and associated hemoglobinopathies.
    • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
    • Sickle Cell Disease: Topic Overview: NorthShore University HealthSystem 10 January 2010 1:26 UTC www.northshore.org [Source type: Academic]
    • CIGNA - Blood transfusions for sickle cell disease 10 January 2010 1:26 UTC www.cigna.com [Source type: Academic]
    • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

    ^ Pulmonary hypertension in sickle cell disease.
    • Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia -- Gladwin and Kato 2005 (1): 51 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

    .Regular annual eye checks are recommended.
  • During pregnancy, intrauterine growth retardation, spontaneous abortion, and pre-eclampsia.
  • Chronic pain: Even in the absence of acute vaso-occlusive pain, many patients have chronic pain that is not reported[13].
  • Pulmonary hypertension (increased pressure on the pulmonary artery), leading to strain on the right ventricle and a risk of heart failure; typical symptoms are shortness of breath, decreased exercise tolerance and episodes of syncope[14].
  • Chronic renal failure due to Sickle cell nephropathy—manifests itself with hypertension (high blood pressure), proteinuria (protein loss in the urine), hematuria (haematuria) (loss of red blood cells in urine) and worsened anaemia.^ Symptoms of sickle cell anemia include: .
    • Sickle Cell Anemia - Sickle Cell Anemia Description - Sickle Cell Anemia Symptoms - Sickle Cell Anemia Prevention - Sickle Cell Anemia Treatment 6 February 2010 13:21 UTC symptomchecker.about.com [Source type: FILTERED WITH BAYES]
    • Pri-Med Patient Education Center - Sickle Cell Anemia 6 February 2010 13:21 UTC www.patienteducationcenter.org [Source type: FILTERED WITH BAYES]

    ^ Over time the heart enlarges, increasing the risk of heart attack and heart failure.
    • Sickle-cell Anemia - Keyword, What Are the Sickle-Cell Trait and Sickle-Cell Disease?, What Are the Effects of Sickle-Cell Disease?, Crises, Anemia 19 September 2009 5:14 UTC www.humanillnesses.com [Source type: FILTERED WITH BAYES]

    ^ Pulmonary hypertension in sickle cell disease.
    • Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia -- Gladwin and Kato 2005 (1): 51 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

    .If it progresses to end-stage renal failure, it carries a poor prognosis.^ If it progresses to end-stage renal failure, it carries a poor prognosis.

    ^ If it progresses to end-stage renal failure it carries a poor prognosis.
    • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

    ^ End-stage Renal Disease .
    • Sickle Cell Anemia - Symptoms, Treatment and Prevention 6 February 2010 13:21 UTC www.healthscout.com [Source type: Academic]

    [15]

Heterozygotes

.The heterozygous form (sickle cell trait) is almost always asymptomatic, and the only usual significant manifestation is the renal concentrating defect presenting with isosthenuria.^ Sickle cell trait is different.
  • Sickle Cell Information Center News Page 19 September 2009 5:14 UTC www.scinfo.org [Source type: Academic]

^ Sickling particularly occurs in renal medulla and most of the sickle cell traits have disturbed renal concentration.
  • ISPUB - Anaesthetic Management Of A Case With Sickle Cell Anemia Undergoing Coronary Artery Bypass Grafting 6 February 2010 13:21 UTC www.ispub.com [Source type: Academic]

^ This is referred to as having the sickle cell trait.
  • dreddyclinic.com - Sickle cell anemia - Sickle cell disease (SCD) 6 February 2010 13:21 UTC www.dreddyclinic.com [Source type: Academic]

Diagnosis

.In HbSS, the full blood count reveals haemoglobin levels in the range of 6–8 g/dL with a high reticulocyte count (as the bone marrow compensates for the destruction of sickle cells by producing more red blood cells).^ The bone marrow slows down and much needed red blood cells are not produced.
  • NJDHSS - Sickle Cell Disease 10 January 2010 1:26 UTC www.nj.gov [Source type: Academic]

^ Barriers to bone marrow transplantation for sickle cell anemia.
  • Journal of Clinical Investigation -- Sickle cell disease: old discoveries, new concepts, and future promise 10 January 2010 1:26 UTC www.jci.org [Source type: Academic]
  • sickle cell anemia - blockage of blood vessels caused by abnormal crescent shaped red blood cells - MEDgle - search your symptoms, diagnoses, drug, procedures, physicians 6 February 2010 13:21 UTC www.medgle.com [Source type: Academic]

^ Multicenter investigation of bone marrow transplantation for sickle cell disease.
  • Sickle Cell Disease -- GeneReviews -- NCBI Bookshelf 10 January 2010 1:26 UTC www.ncbi.nlm.nih.gov [Source type: Academic]
  • Prevention and Management of Stroke in Sickle Cell Anemia -- Platt 2006 (1): 54 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

.In other forms of sickle-cell disease, Hb levels tend to be higher.^ Sickle cell disease and associated hemoglobinopathies.
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Sickle Cell Disease: Topic Overview: NorthShore University HealthSystem 10 January 2010 1:26 UTC www.northshore.org [Source type: Academic]
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Hb AS: Sickle cell trait.

^ Pulmonary hypertension in sickle cell disease.
  • Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia -- Gladwin and Kato 2005 (1): 51 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

.A blood film may show features of hyposplenism (target cells and Howell-Jolly bodies).^ Sickle cells, nucleated red blood cells, and target cells may be seen.
  • Sickle Cell Disease -- GeneReviews -- NCBI Bookshelf 10 January 2010 1:26 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ A blood film may show features of hyposplenism (target cells and Howell-Jolly bodies).

^ Presence of Howell-Jolly bodies indicates hyposplenism.
  • Sickle Cell Disease -- GeneReviews -- NCBI Bookshelf 10 January 2010 1:26 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Sickling of the red blood cells, on a blood film, can be induced by the addition of sodium metabisulfite.^ Important in red blood cell production.
  • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]
  • MoonDragon's ObGyn Information: Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: FILTERED WITH BAYES]

^ Irregular red blood cell shape .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ The disease affects the red blood cells.
  • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 10 January 2010 1:26 UTC www.chop.edu [Source type: Academic]
  • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 6 February 2010 13:21 UTC www.chop.edu [Source type: Academic]

.The presence of sickle haemoglobin can also be demonstrated with the "sickle solubility test". A mixture of haemoglobin S (Hb S) in a reducing solution (such as sodium dithionite) gives a turbid appearance, whereas normal Hb gives a clear solution.^ The presence of sickle haemoglobin can also be demonstrated with the "sickle solubility test".

^ A mixture of haemoglobin S (Hb S) in a reducing solution (such as sodium dithionite) gives a turbid appearance, whereas normal Hb gives a clear solution.

^ Sickle solubility test: a mixture of HbS in a reducing solution such as sodium dithionite gives a turbid appearance because of precipitation of HbS, whereas normal haemoglobin gives a clear solution.
  • Sickle Cell Disease and Sickle Cell Anaemia | Doctor | Patient UK 10 January 2010 1:26 UTC www.patient.co.uk [Source type: FILTERED WITH BAYES]

.Abnormal haemoglobin forms can be detected on haemoglobin electrophoresis, a form of gel electrophoresis on which the various types of haemoglobin move at varying speeds.^ Abnormal haemoglobin forms can be detected on haemoglobin electrophoresis, a form of gel electrophoresis on which the various types of haemoglobin move at varying speeds.

^ Abnormal haemoglobin forms can be detected on haemoglobin electrophoresis, a form of gel electrophoresis on which the various types of haemoglobin move at varying speed.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ This test measures the speed at which a molecule moves in a gel and can detect abnormal hemoglobin HbS. .
  • Sickle Cell Anemia - baby, symptoms, meaning, Definition, Description, Demographics, Causes and symptoms, Diagnosis, Treatment, Prognosis, Prevention 6 February 2010 13:21 UTC www.healthofchildren.com [Source type: Academic]

.Sickle-cell haemoglobin (HgbS) and haemoglobin C with sickling (HgbSC)—the two most common forms—can be identified from there.^ The most common variations of the sickle cell gene include the following: .
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.umm.edu [Source type: Academic]
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.healthsystem.virginia.edu [Source type: Academic]
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.health.uab.edu [Source type: Academic]
  • Norwood Hospital - Sickle Cell Disease 10 January 2010 1:26 UTC www.caritasnorwood.org [Source type: Academic]
  • Sickle Cell Disease - New York Presbyterian Hospital 10 January 2010 1:26 UTC nyp.org [Source type: Academic]
  • Akron General Medical Center -- Sickle Cell Disease 10 January 2010 1:26 UTC healthinfo.akrongeneral.org [Source type: Academic]
  • Sickle Cell Disease - Hematology and Blood Disorders - The Nebraska Medical Center 10 January 2010 1:26 UTC www.nebraskamed.com [Source type: Academic]
  • Sickle Cell Disease 10 January 2010 1:26 UTC healthlibrary.brighamandwomens.org [Source type: Academic]
  • Sickle Cell Disease - University of Chicago Medical Center 10 January 2010 1:26 UTC www.uchospitals.edu [Source type: Academic]
  • OHSU Health Information: Sickle Cell Disease 10 January 2010 1:26 UTC www.ohsu.edu [Source type: Academic]
  • http://medicalcenter.osu.edu/patientcare/healthcare_services/blood_disorders/blood_disorders/anemias/anemia_overiew/sickle_cell_disease/Pages/index.aspx 10 January 2010 1:26 UTC medicalcenter.osu.edu [Source type: Academic]
  • Hematology & Blood Disorders > Sickle Cell Disease (The Methodist Hospital, Houston, Texas) 6 February 2010 13:21 UTC www.methodisthealth.com [Source type: Academic]

^ Painful events are the most common symptom of sickle cell disease.
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Evansville,IN Sickle Cell Disease; Sickle cell disease changes normal, ... 10 January 2010 1:26 UTC www.stmarys.org [Source type: Academic]
  • The University of Kansas Hospital - Sickle Cell Disease 10 January 2010 1:26 UTC www.kumed.com [Source type: Academic]
  • Sickle Cell Disease: Topic Overview: NorthShore University HealthSystem 10 January 2010 1:26 UTC www.northshore.org [Source type: Academic]
  • Sickle Cell Disease - Topic Overview - Yahoo! Health 19 September 2009 5:14 UTC health.yahoo.com [Source type: FILTERED WITH BAYES]

^ What are common characteristics of a sickle cell ulcer?

.The diagnosis can be confirmed with high-performance liquid chromatography (HPLC).^ The diagnosis can be confirmed with high-performance liquid chromatography (HPLC).

^ The diagnosis can be confirmed with high performance liquid chromatography (HPLC).
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ High-performance liquid chromatography (HPLC) .
  • Sickle Cell Disease -- GeneReviews -- NCBI Bookshelf 10 January 2010 1:26 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

Genetic testing is rarely performed, as other investigations are highly specific for HbS and HbC.[16]
.An acute sickle-cell crisis is often precipitated by infection.^ Acute attacks can be confused with a sickle cell crisis in the liver.
  • Sickle cell disease: the causes, diagnosis, and treatment of sickle ... 10 January 2010 1:26 UTC www.henryfordhealth.org [Source type: Academic]
  • Sickle cell disease - Edward Hospital, Naperville IL Area 10 January 2010 1:26 UTC www.edward.org [Source type: Academic]
  • Sickle cell disease 10 January 2010 1:26 UTC www.bcbswny.com [Source type: Academic]
  • Sickle cell disease 10 January 2010 1:26 UTC www.fitsugar.com [Source type: Academic]
  • Sickle Cell Anemia Complications - Sickle Cell Anemia Health Information - NY Times Health 6 February 2010 13:21 UTC health.nytimes.com [Source type: Academic]

^ Vasoocclusive crisis is often triggered by infection.
  • Sickle Cell Anemia: eMedicine Hematology 6 February 2010 13:21 UTC emedicine.medscape.com [Source type: Academic]

^ How to recognise a sickle cell crisis?

.Therefore, a urinalysis to detect an occult urinary tract infection, and chest X-ray to look for occult pneumonia should be routinely performed.^ Treatment of urinary tract infections.
  • Sickle cell disease: the causes, diagnosis, and treatment of sickle ... 10 January 2010 1:26 UTC www.henryfordhealth.org [Source type: Academic]
  • Sickle cell disease - Edward Hospital, Naperville IL Area 10 January 2010 1:26 UTC www.edward.org [Source type: Academic]

^ Therefore, a urinalysis to detect an occult UTI and CXR to look for occult pneumonia should be routinely performed.

^ Urinary tract infection .
  • Fattened by pills - The Boston Globe 6 February 2010 13:21 UTC www.boston.com [Source type: News]

[17]

Pathophysiology

.Sickle-cell anaemia is caused by a point mutation in the β-globin chain of haemoglobin, causing the hydrophilic amino acid glutamic acid to be replaced with the hydrophobic amino acid valine at the sixth position.^ The sickle cell anemia mutation is in the beta chain of hemoglobin.
  • TUTORIAL: Sickle-cell Anemia 19 September 2009 5:14 UTC bioquest.org [Source type: FILTERED WITH BAYES]

^ As a result of this mutation, valine (a non-polar amino acid) is inserted into the b globin chain instead of glutamic acid (an electrically charged amino acid).

^ "Sickle cell anaemia trait".
  • Sickle Cell Patients Resource - www.sicklecell.md 10 January 2010 1:26 UTC www.sicklecell.md [Source type: Academic]

.The β-globin gene is found on the short arm of chromosome 11.^ The β-globin gene is found on the short arm of chromosome 11 .
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ The β globin chain is coded on the short arm of chromosome 11.

^ Sickle cell anaemia results from substitution of a single amino acid, namely valine for glutamic acid in the beta globin gene at chromosome number 11.
  • Sickle cell disease 10 January 2010 1:26 UTC www.rcsed.ac.uk [Source type: Academic]

.The association of two wild-type α-globin subunits with two mutant β-globin subunits forms haemoglobin S (HbS).^ The association of two wild-type α-globin subunits with two mutant β-globin subunits forms haemoglobin S (HbS).

^ Hemoglobin is a complex of four subunits; two of these subunits are produced from birth to death, whereas the other pair is first comprised of a fetal subunit type that switches after birth to an adult form.
  • New Hope for Sickle-Cell Anemia Sufferers: Scientific American 6 February 2010 13:21 UTC www.scientificamerican.com [Source type: Academic]

^ Abnormal haemoglobin forms can be detected on haemoglobin electrophoresis, a form of gel electrophoresis on which the various types of haemoglobin move at varying speeds.

.Under low-oxygen conditions (being at high altitude, for example), the absence of a polar amino acid at position six of the β-globin chain promotes the non-covalent polymerisation (aggregation) of haemoglobin, which distorts red blood cells into a sickle shape and decreases their elasticity.^ The red blood cell in which the haemoglobin is contained also changes its shape, from round to crescent (sickle shaped).

^ As a result of this mutation, valine (a non-polar amino acid) is inserted into the b globin chain instead of glutamic acid (an electrically charged amino acid).

^ Important in red blood cell production.
  • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]
  • MoonDragon's ObGyn Information: Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: FILTERED WITH BAYES]

.The loss of red blood cell elasticity is central to the pathophysiology of sickle-cell disease.^ Sickle cell disease and associated hemoglobinopathies.
  • Blood transfusions for sickle cell disease - Yahoo! Health 15 September 2009 2:51 UTC health.yahoo.com [Source type: Academic]
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Sickle Cell Disease: Topic Overview: NorthShore University HealthSystem 10 January 2010 1:26 UTC www.northshore.org [Source type: Academic]
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Pulmonary hypertension in sickle cell disease.
  • Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia -- Gladwin and Kato 2005 (1): 51 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

^ Splenic sequestration and sickle cell disease .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

.Normal red blood cells are quite elastic, which allows the cells to deform to pass through capillaries.^ This would cause the body to make normal red blood cells.
  • Sickle Cell Anemia, Treatments 15 September 2009 2:51 UTC www.nhlbi.nih.gov [Source type: Academic]
  • Sickle Cell Anemia, All Sections 15 September 2009 2:51 UTC www.nhlbi.nih.gov [Source type: FILTERED WITH BAYES]

^ Normal red blood cells contain hemoglobin A. Hemoglobin S and hemoglobin C are abnormal types of hemoglobin.
  • Sickle Cell Disease | ebonywoman.info 10 January 2010 1:26 UTC ebonywoman.info [Source type: FILTERED WITH BAYES]

^ Important in red blood cell production.
  • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]
  • MoonDragon's ObGyn Information: Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: FILTERED WITH BAYES]

.In sickle-cell disease, low-oxygen tension promotes red blood cell sickling and repeated episodes of sickling damage the cell membrane and decrease the cell's elasticity.^ Sickle cell disease and associated hemoglobinopathies.
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Sickle Cell Disease: Topic Overview: NorthShore University HealthSystem 10 January 2010 1:26 UTC www.northshore.org [Source type: Academic]
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Pulmonary hypertension in sickle cell disease.
  • Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia -- Gladwin and Kato 2005 (1): 51 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

^ Splenic sequestration and sickle cell disease .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

.These cells fail to return to normal shape when normal oxygen tension is restored.^ These cells fail to return to normal shape when normal oxygen tension is restored.

^ Sickle cell disease results when red blood cells make mostly hemoglobin S instead of the normal hemoglobin A. Normal red blood cells are smooth, round and soft; their shape and softness enable them to move easily through the blood vessels to carry oxygen to all parts of the body.
  • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 10 January 2010 1:26 UTC www.chop.edu [Source type: Academic]
  • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 6 February 2010 13:21 UTC www.chop.edu [Source type: Academic]

^ A mutation of the haemoglobin molecule in the red cell responsible for carrying oxygen to the tissues causes the molecules to form chains when oxygen is removed, and these distort the red cells into the sickle shape.

.As a consequence, these rigid blood cells are unable to deform as they pass through narrow capillaries, leading to vessel occlusion and ischaemia.^ In addition, sickled cells are relatively rigid and cannot pass through narrow blood vessels as readily as normal red cells.
  • SICKLE-CELL DISEASE, 6 February 2010 13:21 UTC www.history.com [Source type: FILTERED WITH BAYES]

^ Sickled cells cannot pass easily through tiny blood vessels.
  • Sickle Cell Anemia 6 February 2010 13:21 UTC dwb.unl.edu [Source type: FILTERED WITH BAYES]

^ These cells are very flexible and move easily through small blood vessels.
  • What is sickle cell disease 10 January 2010 1:26 UTC nips.med-web.com [Source type: General]
  • Sickle Cell Anemia 6 February 2010 13:21 UTC dwb.unl.edu [Source type: FILTERED WITH BAYES]

.The actual anemia of the illness is caused by hemolysis, the destruction of the red cells inside the spleen, because of their misshape.^ Increased red cell destruction, or hemolysis .

^ Hemolytic anemia is an inadequate amount of red blood cells, caused by the rapid destruction of red blood cells in the blood or spleen.
  • Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC www.kosmix.com [Source type: Academic]

^ The decrease in red blood cells causes anemia.
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.umm.edu [Source type: Academic]
  • Sickle Cell Disease - My Child Has - Children's Hospital Boston 10 January 2010 1:26 UTC www.childrenshospital.org [Source type: Academic]
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.lpch.org [Source type: Academic]
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.healthsystem.virginia.edu [Source type: Academic]
  • Sickle Cell Disease - Online Medical Encyclopedia - University of Rochester Medical Center 10 January 2010 1:26 UTC www.urmc.rochester.edu [Source type: Academic]
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.health.uab.edu [Source type: Academic]
  • Norwood Hospital - Sickle Cell Disease 10 January 2010 1:26 UTC www.caritasnorwood.org [Source type: Academic]
  • Sickle Cell Disease - New York Presbyterian Hospital 10 January 2010 1:26 UTC nyp.org [Source type: Academic]
  • Sickle Cell Disease - New York Presbyterian Hospital 10 January 2010 1:26 UTC nyp.org [Source type: Academic]
  • Akron General Medical Center -- Sickle Cell Disease 10 January 2010 1:26 UTC healthinfo.akrongeneral.org [Source type: Academic]
  • Howard County General Hospital - Sickle Cell Disease 10 January 2010 1:26 UTC www.hcgh.org [Source type: Academic]
  • Sickle Cell Disease - Hematology and Blood Disorders - The Nebraska Medical Center 10 January 2010 1:26 UTC www.nebraskamed.com [Source type: Academic]
  • Sickle Cell Disease 10 January 2010 1:26 UTC healthlibrary.brighamandwomens.org [Source type: Academic]
  • Sickle Cell Disease - University of Chicago Medical Center 10 January 2010 1:26 UTC www.uchospitals.edu [Source type: Academic]
  • Sickle Cell Disease - University of Chicago Comer Children's Hospital 10 January 2010 1:26 UTC www.uchicagokidshospital.org [Source type: Academic]
  • OHSU Health Information: Sickle Cell Disease 10 January 2010 1:26 UTC www.ohsu.edu [Source type: Academic]
  • http://medicalcenter.osu.edu/patientcare/healthcare_services/blood_disorders/blood_disorders/anemias/anemia_overiew/sickle_cell_disease/Pages/index.aspx 10 January 2010 1:26 UTC medicalcenter.osu.edu [Source type: Academic]
  • Hematology and Blood Disorders 6 February 2010 13:21 UTC www.rush.edu [Source type: Academic]
  • Hematology & Blood Disorders > Sickle Cell Disease (The Methodist Hospital, Houston, Texas) 6 February 2010 13:21 UTC www.methodisthealth.com [Source type: Academic]

.Although the bone marrow attempts to compensate by creating new red cells, it does not match the rate of destruction.^ It does NOT create new genetic strings.
  • Black African Footprint: Sickle Cell 10 January 2010 1:26 UTC www.white-history.com [Source type: Academic]

^ It replaces diseased bone marrow cells with healthy cells from a matched donor.
  • When Your Child Has Sickle Cell Anemia | HealthSheets | Wellness Library | Mount Nittany Medical Center 6 February 2010 13:21 UTC www.mountnittany.org [Source type: Academic]

^ Bone marrow/stem cell transplantation .
  • Treating Sickle Cell Disease - US News and World Report 10 January 2010 1:26 UTC health.usnews.com [Source type: Academic]

[18] .Healthy red blood cells typically live 90-120 days, but sickle cells only survive 10–20 days.^ Unlike normal red blood cells, which last about 120 days in the bloodstream, sickled red cells die after only 10 to 20 days.
  • Center for Cancer and Blood Disorders > Sickle Cell FAQ 10 January 2010 1:26 UTC www.childrens.com [Source type: Academic]

^ Important in red blood cell production.
  • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]
  • MoonDragon's ObGyn Information: Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: FILTERED WITH BAYES]

^ Irregular red blood cell shape .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

[19]

Genetics

File:Sickle cell haemoglobin.png
A single amino acid change causes haemoglobin proteins to form fibers.
.Sickle-cell gene mutation probably arose spontaneously in different geographic areas, as suggested by restriction endonuclease analysis.^ Sickle cell trait is different.
  • Sickle Cell Information Center News Page 19 September 2009 5:14 UTC www.scinfo.org [Source type: Academic]

^ What are the different types of sickle cell disorders?
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]

^ The variant genes are call sickle cell genes.
  • Sickle Cell Anemia Symptoms 19 September 2009 5:14 UTC www.iifar.org [Source type: Academic]

.These variants are known as Cameroon, Senegal, Benin, Bantu and Saudi-Asian.^ These variants are known as Cameroon, Senegal, Benin, Bantu and Saudi-Asian.

^ These clinically important variants are known as Cameroon, Senegal, Benin, Bantu and Saudi-Asian.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ "Senegal haplotype is associated with higher HbF than Benin and Cameroon haplotypes in African children with sickle cell anemia ".

.Their clinical importance springs from the fact that some of them are associated with higher HbF levels, e.g., Senegal and Saudi-Asian variants, and tend to have milder disease.^ Their clinical importance springs from the fact that some of them are associated with higher HbF levels e.g Senegal and Saudi-Asian variants, and tend to have milder disease.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ Their clinical importance springs from the fact that some of them are associated with higher HbF levels, e.g., Senegal and Saudi-Asian variants, and tend to have milder disease.

^ Thromboelastography assesses the functionality of this system from a global standpoint and has demonstrated some utility in detecting hypercoagulable states in varied clinical settings, but it has not been systematically evaluated in patients with sickle cell disease.

[20]
.In people heterozygous for HgbS (carriers of sickling haemoglobin), the polymerisation problems are minor, because the normal allele is able to produce over 50% of the haemoglobin.^ In people heterozygous for HgbS (carriers of sickling haemoglobin), the polymerisation problems are minor, because the normal allele is able to produce over 50% of the haemoglobin.

^ Haemoglobin H is produced by four normal b chains.

^ In people heterozygous for HgbS ( carriers of sickling haemoglobin), the polymerisation problems are minor.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

.In people homozygous for HgbS, the presence of long-chain polymers of HbS distort the shape of the red blood cell from a smooth donut-like shape to ragged and full of spikes, making it fragile and susceptible to breaking within capillaries.^ This would cause the body to make normal red blood cells.
  • Sickle Cell Anemia, All Sections 15 September 2009 2:51 UTC www.nhlbi.nih.gov [Source type: FILTERED WITH BAYES]

^ Normal red blood cells contain only normal hemoglobin and are shaped like doughnuts.
  • Sickle Cell Anemia 6 February 2010 13:21 UTC dwb.unl.edu [Source type: FILTERED WITH BAYES]

^ Important in red blood cell production.
  • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]
  • MoonDragon's ObGyn Information: Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: FILTERED WITH BAYES]

.Carriers have symptoms only if they are deprived of oxygen (for example, while climbing a mountain) or while severely dehydrated.^ Carriers only have symptoms if they are deprived of oxygen (for example, while climbing a mountain) or while severely dehydrated .
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ Carriers have symptoms only if they are deprived of oxygen (for example, while climbing a mountain) or while severely dehydrated.

^ Because they don’t expand circulation is restricted and oxygen starvation occurs in tissues downstream, causing the pain and other symptoms of sickle cell anemia.
  • Breakthrough For Sickle Cell Anemia 19 September 2009 5:14 UTC www.health-reports.com [Source type: FILTERED WITH BAYES]

.Under normal circumstances, these painful crises occur about 0.8 times per year per patient.^ "These painful crises can occur any time and can last from days to weeks.
  • Sickle Cell Center Opens at Montefiore 10 January 2010 1:26 UTC www.montefiore.org [Source type: Academic]

^ Under normal circumstances, these painful crises occur 0.8 times per year per patient.

^ Normally these painful crises occur 0.8 times per year per patient.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

.November 2008" style="white-space:nowrap;">[citation needed] The sickle-cell disease occurs when the seventh amino acid (if we count the initial methionine), glutamic acid, is replaced by valine to change its structure and function.^ Sickle cell disease and associated hemoglobinopathies.
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Pulmonary hypertension in sickle cell disease.
  • Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia -- Gladwin and Kato 2005 (1): 51 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

^ Splenic sequestration and sickle cell disease .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

Distribution of the sickle-cell trait shown in pink and purple
Historical distribution of malaria (no longer endemic in Europe) shown in green
Modern distribution of malaria
.The gene defect is a known mutation of a single nucleotide (see single-nucleotide polymorphism - SNP) (T to A) of the β-globin gene, which results in glutamate being substituted by valine at position 6. Haemoglobin S with this mutation are referred to as HbS, as opposed to the normal adult HbA. The genetic disorder is due to the mutation of a single nucleotide, from a GAG to GTG codon mutation.^ Distribution of the sickle-cell trait shown in pink and purple Historical distribution of malaria (no longer endemic in Europe) shown in green Modern distribution of malaria The gene defect is a known mutation of a single nucleotide (see single nucleotide polymorphism - SNP) (A to T) of the β-globin gene, which results in glutamate being substituted by valine at position 6.

^ Hb S arises from a mutation substituting thymine for adenine in the sixth codon of the beta-chain gene, GAG to GTG. This causes coding of valine instead of glutamate in position 6 of the Hb beta chain.
  • Sickle Cell Anemia: eMedicine Hematology 6 February 2010 13:21 UTC emedicine.medscape.com [Source type: Academic]

^ In Hb S, valine is substituted for glutamic acid in the 6th amino acid of the β chain.
  • Sickle Cell Disease: Anemias Caused by Hemolysis: Merck Manual Professional 6 February 2010 13:21 UTC www.merck.com [Source type: Academic]

.This is normally a benign mutation, causing no apparent effects on the secondary, tertiary, or quaternary structure of haemoglobin in conditions of normal oxygen concentration.^ This is normally a benign mutation, causing no apparent effects on the secondary, tertiary, or quaternary structure of haemoglobin.

^ This is normally a benign mutation, causing no apparent effects on the secondary , tertiary , or quaternary structure of haemoglobin.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ A single amino acid substitution mutation results in the production of abnormal hemoglobin HbS which polymerizes under conditions of low oxygen saturation and causes red cells to stiffen into a sickle shape.
  • Sickle Cell Disease I � Musculoskeletal System 10 January 2010 1:26 UTC www.ptolemy.ca [Source type: Academic]

.What it does allow for, under conditions of low oxygen concentration, is the polymerization of the HbS itself.^ What it does allow for, under conditions of low oxygen concentration, is the polymerization of the HbS itself.

^ Under low-oxygen conditions (being at high altitude, for example), the absence of a polar amino acid at position six of the β-globin chain promotes the non-covalent polymerisation (aggregation) of haemoglobin, which distorts red blood cells into a sickle shape and decreases their elasticity.

^ The pathophysiology of the disease is complex, but is ultimately due to the beta-globin point mutation that allows HbS polymerization to occur under conditions of hypoxia, acidemia, fever, and erythrocyte dehydration.
  • Modulation of Fetal Hemoglobin in Sickle Cell Anemia 6 February 2010 13:21 UTC cme.medscape.com [Source type: Academic]

.The deoxy form of haemoglobin exposes a hydrophobic patch on the protein between the E and F helices.^ The deoxy form of haemoglobin exposes a hydrophobic patch on the protein between the E and F helices.

^ A single amino acid change causes haemoglobin proteins to form fibers.

^ The amino acids in a protein form associations with each other that try to keep hydrophobic amino acids together and separate them from the water and hydrophilic amino acids.
  • TUTORIAL: Sickle-cell Anemia 19 September 2009 5:14 UTC bioquest.org [Source type: FILTERED WITH BAYES]

.The hydrophobic residues of the valine at position 6 of the beta chain in haemoglobin are able to associate with the hydrophobic patch, causing haemoglobin S molecules to aggregate and form fibrous precipitates.^ The hydrophobic residues of the valine at position 6 of the beta chain in haemoglobin are able to bind to the hydrophobic patch, causing haemoglobin S molecules to aggregate and form fibrous precipitates.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ This causes the association of 2 individual hemoglobin molecules.
  • TUTORIAL: Sickle-cell Anemia 19 September 2009 5:14 UTC bioquest.org [Source type: FILTERED WITH BAYES]

^ The hydrophobic residues of the valine at position 6 of the beta chain in haemoglobin are able to associate with the hydrophobic patch, causing haemoglobin S molecules to aggregate and form fibrous precipitates.

.The allele responsible for sickle-cell anaemia is autosomal recessive and can be found on the short arm of chromosome 11. A person that receives the defective gene from both father and mother develops the disease; a person that receives one defective and one healthy allele remains healthy, but can pass on the disease and is known as a carrier.^ Sickle cell anemia is also known as Hemoglobin S disease.

^ Sickle cell disease and associated hemoglobinopathies.
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Pulmonary hypertension in sickle cell disease.
  • Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia -- Gladwin and Kato 2005 (1): 51 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

.If two parents who are carriers have a child, there is a 1-in-4 chance of their child's developing the disease and a 1-in-2 chance of their child's being just a carrier.^ A carrier has an increased chance to have a child with sickle cell disease.
  • Sickle Cell Disease - My Child Has - Children's Hospital Boston 10 January 2010 1:26 UTC www.childrenshospital.org [Source type: Academic]
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.chw.org [Source type: Academic]
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.healthsystem.virginia.edu [Source type: Academic]
  • Sickle Cell Disease, Robert Wood Johnson University Hospital, New Brunswick, NJ, 08903 10 January 2010 1:26 UTC www.rwjuh.edu [Source type: Academic]

^ If two parents who are carriers have a child, there is a 1-in-4 chance of their child developing the illness and a 1-in-2 chance of their child just being a carrier.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ And there is a 50 percent chance that a child will be a carrier of the disease, like the parents.
  • Departments & Programs - Children's National Medical Center 10 January 2010 1:26 UTC www.childrensnational.org [Source type: Academic]

.Since the gene is incompletely recessive, carriers can produce a few sickled red blood cells, not enough to cause symptoms, but enough to give resistance to malaria.^ Important in red blood cell production.
  • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]
  • MoonDragon's ObGyn Information: Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: FILTERED WITH BAYES]

^ Irregular red blood cell shape .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ The disease affects the red blood cells.
  • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 10 January 2010 1:26 UTC www.chop.edu [Source type: Academic]
  • Diagnosing and treating sickle cell disease | The Children's Hospital of Philadelphia 6 February 2010 13:21 UTC www.chop.edu [Source type: Academic]

.Because of this, heterozygotes have a higher fitness than either of the homozygotes.^ Because of this, heterozygotes have a higher fitness than either of the homozygotes.

^ To date, very few transplantation procedures have been performed in adults with SCD because of concerns that the morbidity and mortality of BMT is higher in adults than in children.
  • Journal of Clinical Investigation -- Sickle cell disease: old discoveries, new concepts, and future promise 10 January 2010 1:26 UTC www.jci.org [Source type: Academic]

^ The homozygotes of sickle cell disease die earlier (younger) than the heterozygotes (trait or carriers).
  • ISPUB - Epidemiology, Population Health Genetics and Phenotypic Diversity of Sickle Cell Disease in India 10 January 2010 1:26 UTC www.ispub.com [Source type: Academic]

.This is known as heterozygote advantage.^ This is known as heterozygote advantage.

^ This is known as heterozygote advantage .
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

.Due to the adaptive advantage of the heterozygote, the disease is still prevalent, especially among people with recent ancestry in malaria-stricken areas, such as Africa, the Mediterranean, India and the Middle East.^ The disease is found predominantly in Afro-Caribbean people; it also occurs in the Middle East and the Mediterranean area.

^ Due to the adaptive advantage of the heterozygote, the disease is still prevalent, especially among people with recent ancestry in malaria -stricken areas, such as Africa, the Mediterranean, India and the Middle East.

^ The prevalence of this disease in Africa, the Mediterranean and the Middle East is attributed to the increased protection to malaria infection in heterozygous carriers.
  • Centre for Arab Genomic Studies (CAGS) --> CTGA --> Sickle Cell Anemia 6 February 2010 13:21 UTC www.cags.org.ae [Source type: Academic]

[21] .Malaria was historically endemic to southern Europe, but it was declared eradicated in the mid-20th century, with the exception of rare sporadic cases.^ Malaria was historically endemic to southern Europe, but it was declared eradicated in the mid-20th century, with the exception of rare sporadic cases.

[22][23]
.The Price equation is a simplified mathematical model of the genetic evolution of sickle-cell anaemia.^ Sickle cell anemia is a genetic disorder.

^ Henderson AB. Sickle cell anaemia.
  • Sickle cell disease 10 January 2010 1:26 UTC www.rcsed.ac.uk [Source type: Academic]

^ Sickle cell (HbSS) is a genetic disease.
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.chw.org [Source type: Academic]
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.healthsystem.virginia.edu [Source type: Academic]
  • Sickle Cell Disease, Robert Wood Johnson University Hospital, New Brunswick, NJ, 08903 10 January 2010 1:26 UTC www.rwjuh.edu [Source type: Academic]

.The malaria parasite has a complex life cycle and spends part of it in red blood cells.^ Important in red blood cell production.
  • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]
  • MoonDragon's ObGyn Information: Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: FILTERED WITH BAYES]

^ Irregular red blood cell shape .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ The malaria parasite has a complex life cycle and spends part of it in red blood cells.

.In a carrier, the presence of the malaria parasite causes the red blood cells with defective haemoglobin to rupture prematurely, making the plasmodium unable to reproduce.^ This would cause the body to make normal red blood cells.
  • Sickle Cell Anemia, All Sections 15 September 2009 2:51 UTC www.nhlbi.nih.gov [Source type: FILTERED WITH BAYES]

^ Important in red blood cell production.
  • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]
  • MoonDragon's ObGyn Information: Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: FILTERED WITH BAYES]

^ Irregular red blood cell shape .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

.Further, the polymerization of Hb affects the ability of the parasite to digest Hb in the first place.^ Further, the polymerization of Hb affects the ability of the parasite to digest Hb in the first place.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ At first, HbS retains its ability to return to its soluble form, and red cells can regain their elasticity upon reoxygenation.
  • Sickle Cell Anemia: eMedicine Pediatrics: General Medicine 6 February 2010 13:21 UTC emedicine.medscape.com [Source type: Academic]

^ Atweh further noted that HbF has been shown in vitro to inhibit HbS polymerization via 2 primary mechanisms.
  • Modulation of Fetal Hemoglobin in Sickle Cell Anemia 6 February 2010 13:21 UTC cme.medscape.com [Source type: Academic]

.Therefore, in areas where malaria is a problem, people's chances of survival actually increase if they carry sickle-cell trait (selection for the heterozygote).^ Sickle cell trait is different.
  • Sickle Cell Information Center News Page 19 September 2009 5:14 UTC www.scinfo.org [Source type: Academic]

^ This is referred to as having the sickle cell trait.
  • dreddyclinic.com - Sickle cell anemia - Sickle cell disease (SCD) 6 February 2010 13:21 UTC www.dreddyclinic.com [Source type: Academic]

^ Instead, you will have sickle cell trait.
  • Sickle Cell Anemia, All Sections 15 September 2009 2:51 UTC www.nhlbi.nih.gov [Source type: FILTERED WITH BAYES]

.In the USA, where there is no endemic malaria, the prevalence of sickle-cell anaemia among blacks is lower (about 0.25%) than in West Africa (about 4.0%) and is falling.^ Learning about sickle cell disease: .
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Evansville,IN Sickle Cell Disease; Sickle cell disease changes normal, ... 10 January 2010 1:26 UTC www.stmarys.org [Source type: Academic]

^ "Sickle cell anaemia trait".
  • Sickle Cell Patients Resource - www.sicklecell.md 10 January 2010 1:26 UTC www.sicklecell.md [Source type: Academic]

^ "Why is Sickle Cell Anaemia only found in Black people?"
  • Sickle Cell Patients Resource - www.sicklecell.md 10 January 2010 1:26 UTC www.sicklecell.md [Source type: Academic]

.Without endemic malaria from Africa, the sickle cell mutation is purely disadvantageous and will tend to be selected out of the affected population.^ Mortality from sickle cell disease in Africa.
  • NEJM -- Mortality In Sickle Cell Disease -- Life Expectancy and Risk Factors for Early Death 6 February 2010 13:21 UTC content.nejm.org [Source type: Academic]

^ Who is affected by sickle cell disease?
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Evansville,IN Sickle Cell Disease; Sickle cell disease changes normal, ... 10 January 2010 1:26 UTC www.stmarys.org [Source type: Academic]
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.healthsystem.virginia.edu [Source type: Academic]
  • Sickle Cell Disease, Robert Wood Johnson University Hospital, New Brunswick, NJ, 08903 10 January 2010 1:26 UTC www.rwjuh.edu [Source type: Academic]

^ Without endemic malaria from Africa, the condition is purely disadvantageous, and will tend to be bred out of the affected population.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

.Another factor limiting the spread of sickle-cell genes in North America is the absence of cultural proclivities to polygamy.^ It is believed that at least 8% of blacks in North America carry the gene for sickle cell disease.
  • Sickle Cell Anemia 19 September 2009 5:14 UTC www.ringsurf.com [Source type: Academic]

^ Another factor limiting the spread of sickle-cell genes in North America is the absence of cultural proclivities to polygamy.

^ Evolution of the defective sickle cell gene .
  • Sickle Cell Anemia 19 September 2009 5:14 UTC www.ringsurf.com [Source type: Academic]

[24]
Sickle-cell disease is inherited in the autosomal recessive pattern.

Inheritance

  • Sickle-cell conditions are inherited from parents in much the same way as blood type, hair color and texture, eye color, and other physical traits.
  • The types of haemoglobin a person makes in the red blood cells depend on what haemoglobin genes are inherited from his parents.
  1. If one parent has sickle-cell anaemia (SS) and the other has sickle-cell trait (AS), there is a 50% chance of a child's having sickle-cell disease (SS) and a 50% chance of a child's having sickle-cell trait (AS).
  2. When both parents have sickle-cell trait (AS), a child has a 25% chance (1 of 4) of sickle-cell disease (SS), as shown in the diagram.

Treatment

Folic acid and penicillin

.Children born with sickle-cell disease will undergo close observation by the pediatrician and will require management by a hematologist to assure they remain healthy.^ Sickle cell disease and associated hemoglobinopathies.
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Pulmonary hypertension in sickle cell disease.
  • Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia -- Gladwin and Kato 2005 (1): 51 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

^ Splenic sequestration and sickle cell disease .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

.These patients will take a 1 mg dose of folic acid daily for life.^ Patients should take daily folic acid and vitamin B12 and B6 supplements.
  • Sickle cell disease: the causes, diagnosis, and treatment of sickle ... 10 January 2010 1:26 UTC www.henryfordhealth.org [Source type: Academic]
  • Sickle cell disease 10 January 2010 1:26 UTC www.bcbswny.com [Source type: Academic]
  • Sickle cell disease 10 January 2010 1:26 UTC www.fitsugar.com [Source type: Academic]

^ Take folic acid supplements daily, and eat a balanced diet.
  • Sickle cell anemia - Valley Health System 6 February 2010 13:21 UTC www.valleyhealth.com [Source type: FILTERED WITH BAYES]
  • Sickle cell anemia 6 February 2010 13:21 UTC www.cnn.com [Source type: FILTERED WITH BAYES]
  • MayoClinic.com Health Library - Sickle cell anemia 6 February 2010 13:21 UTC www.riversideonline.com [Source type: FILTERED WITH BAYES]
  • Sickle cell anemia 6 February 2010 13:21 UTC www.studentnews.cnn.com [Source type: FILTERED WITH BAYES]
  • Sickle cell anemia 19 September 2009 5:14 UTC cnn.com [Source type: FILTERED WITH BAYES]

^ Be sure to discuss these with your doctor: Take daily folic acid supplements.

.From birth to five years of age, they will also have to take penicillin daily due to the immature immune system that makes them more prone to early childhood illnesses.^ Daily penicillin up to age five.
  • Sickle Cell Anemia - Medpedia 6 February 2010 13:21 UTC wiki.medpedia.com [Source type: Academic]

^ Daily antibiotics from 2 months to 5 years of age.
  • Evansville,IN Sickle Cell Disease; Sickle cell disease changes normal, ... 10 January 2010 1:26 UTC www.stmarys.org [Source type: Academic]

^ Increase penicillin to 250 mg twice daily from 3 years of age.
  • Sickle Cell Disease in Childhood: Part I. Laboratory Diagnosis, Pathophysiology and Health Maintenance - September 1, 2000 - American Family Physician 10 January 2010 1:26 UTC www.aafp.org [Source type: Academic]

Painful (vaso-occlusive) crisis

.Most people with sickle-cell disease have intensely painful episodes called vaso-occlusive crises.^ Sickle cell disease and associated hemoglobinopathies.
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Special needs of people living with sickle cell disease .
  • Evansville,IN Sickle Cell Disease; Sickle cell disease changes normal, ... 10 January 2010 1:26 UTC www.stmarys.org [Source type: Academic]

^ Splenic sequestration and sickle cell disease .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

.The frequency, severity, and duration of these crises, however, vary tremendously.^ The frequency, severity, and duration of these crises varies considerably.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ The frequency, severity, and duration of these crises, however, vary tremendously.

^ The frequency and duration of the pain can vary tremendously from person to person and over an individual's lifecycle.
  • Sickle Cell Disease - Definition, Description, Causes and symptoms, Diagnosis, Treatment, Psychosocial support, Prognosis 10 January 2010 1:26 UTC health.stateuniversity.com [Source type: Academic]

.Painful crises are treated symptomatically with analgesics; pain management requires opioid administration at regular intervals until the crisis has settled.^ Painful crises are treated with hydration and analgesics; pain management requires opioid administration at regular intervals until the crisis has settled.

^ Painful crises are treated symptomatically with analgesics ; pain management requires opioid administration at regular intervals until the crisis has settled.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ Painful crises are treated symptomatically with analgesics; pain management requires opioid administration at regular intervals until the crisis has settled.

.For milder crises, a subgroup of patients manage on NSAIDs (such as diclofenac or naproxen).^ For milder crises a subgroup of patients manage on NSAIDs (such as diclofenac or naproxen ).
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ For milder crises, a subgroup of patients manage on NSAIDs (such as diclofenac or naproxen).

^ Some patients with severe symptoms receive regular blood transfusions to prevent crises and/or other complications such as stroke and organ damage.
  • Sickle Cell Anemia - baby, symptoms, meaning, Definition, Description, Demographics, Causes and symptoms, Diagnosis, Treatment, Prognosis, Prevention 6 February 2010 13:21 UTC www.healthofchildren.com [Source type: Academic]

.For more severe crises, most patients require inpatient management for intravenous opioids; patient-controlled analgesia (PCA) devices are commonly used in this setting.^ Older patients with severe pain may also require intravenous administration.
  • Sickle cell disease - Edward Hospital, Naperville IL Area 10 January 2010 1:26 UTC www.edward.org [Source type: Academic]
  • Sickle cell disease 10 January 2010 1:26 UTC www.bcbswny.com [Source type: Academic]

^ For more severe crises most patients require inpatient management for intravenous opioids; patient-controlled analgesia (PCA) devices are commonly used in this setting.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ For more severe crises, most patients require inpatient management for intravenous opioids; patient-controlled analgesia (PCA) devices are commonly used in this setting.

.Diphenhydramine is also an effective agent that is frequently prescribed by doctors in order to help control any itching associated with the use of opioids.^ Diphenhydramine is sometimes effective for the itching associated with the opioid use.

^ Diphenhydramine is also an effective agent that is frequently prescribed by doctors in order to help control any itching associated with the use of opioids.

^ Diphenhydramine is effective for the itching associated with the opioid use.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

Acute chest crisis

.Management is similar to vaso-occlusive crisis, with the addition of antibiotics (usually a quinolone or macrolide, since wall-deficient ["atypical"] bacteria are thought to contribute to the syndrome),[25] oxygen supplementation for hypoxia, and close observation.^ Vaso-occlusive crisis .

^ Management is similar to vaso-occlusive crisis, with the addition of antibiotics (usually a quinolone or macrolide, since wall-deficient ["atypical"] bacteria are thought to contribute to the syndrome), [28] oxygen supplementation for hypoxia, and close observation.

^ But they may develop vaso-occlusive crisis, which may lead to hypoxia.

.Should the pulmonary infiltrate worsen or the oxygen requirements increase, simple blood transfusion or exchange transfusion is indicated.^ Blood exchange decreases blood viscosity unlike simple transfusions.
  • ISPUB - Anaesthetic Management Of A Case With Sickle Cell Anemia Undergoing Coronary Artery Bypass Grafting 6 February 2010 13:21 UTC www.ispub.com [Source type: Academic]

^ Should the pulmonary infiltrate worsen or the oxygen requirements increase, simple blood transfusion or exchange transfusion is indicated.

^ Hospitalization and a blood transfusion may be required.
  • NJDHSS - Sickle Cell Disease 10 January 2010 1:26 UTC www.nj.gov [Source type: Academic]

.The latter involves the exchange of a significant portion of the patients red cell mass for normal red cells, which decreases the percent of haemoglobin S in the patient's blood.^ This would cause the body to make normal red blood cells.
  • Sickle Cell Anemia, All Sections 15 September 2009 2:51 UTC www.nhlbi.nih.gov [Source type: FILTERED WITH BAYES]

^ Important in red blood cell production.
  • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]
  • MoonDragon's ObGyn Information: Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: FILTERED WITH BAYES]

^ Irregular red blood cell shape .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

Hydroxyurea

.The first approved drug for the causative treatment of sickle-cell anaemia, hydroxyurea, was shown to decrease the number and severity of attacks in a study in 1995 (Charache et al.)[26] and shown to possibly increase survival time in a study in 2003 (Steinberg et al.).^ The severity of sickle cell disease generally depends on a number of factors: .
  • Sickle cell disease: the causes, diagnosis, and treatment of sickle ... 10 January 2010 1:26 UTC www.henryfordhealth.org [Source type: Academic]
  • Sickle cell disease - Edward Hospital, Naperville IL Area 10 January 2010 1:26 UTC www.edward.org [Source type: Academic]
  • Sickle cell disease 10 January 2010 1:26 UTC www.bcbswny.com [Source type: Academic]
  • Sickle cell disease 10 January 2010 1:26 UTC www.fitsugar.com [Source type: Academic]

^ Barriers to hydroxyurea treatment are shown in table 4.
  • Hydroxyurea Treatment for Sickle Cell Disease 10 January 2010 1:26 UTC consensus.nih.gov [Source type: Academic]

^ "Sickle cell anaemia trait".
  • Sickle Cell Patients Resource - www.sicklecell.md 10 January 2010 1:26 UTC www.sicklecell.md [Source type: Academic]

[27] .This is achieved, in part, by reactivating fetal haemoglobin production in place of the haemoglobin S that causes sickle-cell anaemia.^ Product ratings for sickle cell anemia .
  • Sickle Cell Anemia 10 January 2010 1:26 UTC www.publix.com [Source type: Academic]
  • Sickle Cell Anemia 10 January 2010 1:26 UTC www.nutritionfocus.org [Source type: Academic]
  • Health Library - 10 January 2010 1:26 UTC myhealth.ucsd.edu [Source type: Academic]
  • Trinity, Texas - Sickle Cell Anemia; Sickle cell anemia may result in vitamin ... 6 February 2010 13:21 UTC www.tmfhs.org [Source type: Academic]
  • Sickle Cell Anemia 6 February 2010 13:21 UTC www.naturalmedicine.com [Source type: Academic]
  • Citizens Health Information 6 February 2010 13:21 UTC www.citizensmemorial.com [Source type: Academic]
  • Sickle Cell Anemia | Natural Path 6 February 2010 13:21 UTC www.naturalpath.com [Source type: Academic]
  • The Vitamin Shoppe 6 February 2010 13:21 UTC www.vitaminshoppe.com [Source type: Academic]
  • Join Truestar now and achieve total health and weight loss now! 6 February 2010 13:21 UTC www.truestarhealth.com [Source type: Academic]
  • Delicious Living - for the organic, green and healthy lifestyle 6 February 2010 13:21 UTC healthnotes.deliciouslivingmag.com [Source type: Academic]

^ "Sickle cell anaemia trait".
  • Sickle Cell Patients Resource - www.sicklecell.md 10 January 2010 1:26 UTC www.sicklecell.md [Source type: Academic]

^ What causes sickle cell disease?
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Evansville,IN Sickle Cell Disease; Sickle cell disease changes normal, ... 10 January 2010 1:26 UTC www.stmarys.org [Source type: Academic]
  • Sickle Cell Disease, Robert Wood Johnson University Hospital, New Brunswick, NJ, 08903 10 January 2010 1:26 UTC www.rwjuh.edu [Source type: Academic]
  • Sickle Cell Anemia 6 February 2010 13:21 UTC dwb.unl.edu [Source type: FILTERED WITH BAYES]

.Hydroxyurea had previously been used as a chemotherapy agent, and there is some concern that long-term use may be harmful, but this risk has been shown to be either absent or very small and it is likely that the benefits outweigh the risks.^ Hydroxyurea had previously been used as a chemotherapy agent, and there is some concern that long-term use may be harmful, but this risk has been shown to be either absent or very small and it is likely that the benefits outweigh the risks.

^ What are the short– and long–term harms of hydroxyurea treatment?
  • Hydroxyurea Treatment for Sickle Cell Disease 10 January 2010 1:26 UTC consensus.nih.gov [Source type: Academic]

^ What are the short- and long-term harms of hydroxyurea treatment?
  • National Institutes of Health Consensus Development Conference Statement: Hydroxyurea Treatment for Sickle Cell Disease — Ann Intern Med 10 January 2010 1:26 UTC www.annals.org [Source type: Academic]

[28]

Bone marrow transplants

.Bone marrow transplants have proven to be effective in children.^ Bone marrow transplantation .
  • Sickle Cell Disease - Definition, Description, Causes and symptoms, Diagnosis, Treatment, Psychosocial support, Prognosis 10 January 2010 1:26 UTC health.stateuniversity.com [Source type: Academic]
  • Sickle Cell Anemia - Medpedia 6 February 2010 13:21 UTC wiki.medpedia.com [Source type: Academic]

^ Bone marrow transplants .

^ Bone marrow transplants have proven to be effective in children.

[29]

Future treatments

.Various approaches are being sought for preventing sickling episodes as well as for the complications of sickle-cell disease.^ Sickle cell disease and associated hemoglobinopathies.
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Pulmonary hypertension in sickle cell disease.
  • Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia -- Gladwin and Kato 2005 (1): 51 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

^ Splenic sequestration and sickle cell disease .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

.Other ways to modify haemoglobin switching are being investigated, including the use of phytochemicals such as nicosan.^ Other ways to modify hemoglobin switching are being investigated, including the use of phytochemicals such as nicosan .
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ Other ways to modify hemoglobin switching are being investigated, including the use of phytochemicals such as nicosan.

^ Clinical investigators started to look at these "unexplained bright objects," as they were originally called, in a systematic way and found that they occurred in ~20% of SS children—more or less dependent on the sensitivity of the MRI being used.

.Gene therapy is under investigation.^ Gene therapy is under investigation.

^ Gene therapy offers hope for a cure, but it is still under study.
  • Sickle Cell Disease: Anemias Caused by Hemolysis: Merck Manual Professional 6 February 2010 13:21 UTC www.merck.com [Source type: Academic]

^ Therapies Under Investigation .
  • Sickle Cell Disease -- GeneReviews -- NCBI Bookshelf 10 January 2010 1:26 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

Situation of carriers

.People who are known carriers of the disease often undergo genetic counseling before they have a child.^ People who are known carriers of the disease often undergo genetic counseling before they have a child.

^ People who have the disease are born with it.
  • Sickle Cell Anemia, All Sections 15 September 2009 2:51 UTC www.nhlbi.nih.gov [Source type: FILTERED WITH BAYES]

^ Sickle cell trait is a genetic state, not a disease, and it cannot develop into SCD. Having the sickle cell trait does, however, increase the odds of producing a child with two copies of the hemoglobin S gene, who therefore who has sickle cell disease.
  • About Sickle Cell Disease - US News and World Report 10 January 2010 1:26 UTC health.usnews.com [Source type: FILTERED WITH BAYES]

.A test to see if an unborn child has the disease takes either a blood sample from the fetus or a sample of amniotic fluid.^ Your child will need to see the doctor often for blood tests .
  • Sickle Cell Anemia, All Sections 15 September 2009 2:51 UTC www.nhlbi.nih.gov [Source type: FILTERED WITH BAYES]

^ A test to see if an unborn child has the disease takes either a blood sample from the unborn or a sample of amniotic fluid .
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ Since taking a blood sample from a fetus has risks, the latter test is usually used.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

.Since taking a blood sample from a fetus has greater risks, the latter test is usually used.^ Since taking a blood sample from a fetus has risks, the latter test is usually used.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ Diagnosing Sickle Cell Disease Doctors use a simple blood test to diagnose sickle cell disease.
  • Caregivers: Sickle Cell Disease - NetofCare 10 January 2010 1:26 UTC www.netofcare.org [Source type: Academic]
  • Pain Medicine & Palliative Care 19 September 2009 5:14 UTC www.wehealny.org [Source type: Academic]

^ States that test for sickle cell disorders as part of their newborn screening procedures usually confirm the original test result by retesting the child and will also request a repeat specimen if the history indicates that the child was transfused before the blood sample was obtained.
  • MANAGEMENT AND THERAPY OF SICKLE CELL DISEASE, NIH PUB # 95-2117 10 January 2010 1:26 UTC research.downstate.edu [Source type: Academic]

.After the mutation responsible for this disease was discovered in 1979, the U.S. Air Force required black applicants to test for the mutation.^ After the mutation responsible for this disease was discovered in 1979 , the U.S. Air Force required African American applicants to test for the mutation.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ After the mutation responsible for this disease was discovered in 1979, the U.S. Air Force required black applicants to test for the mutation.

^ The National Institutes of Health recommends that all newborns be screened for sickle cell disease, and testing at birth is now required in almost every state.
  • MySpace - Sickle Cell - 25 - Mujer - COLUMBUS - myspace.com/sickle_cell_anemia 6 February 2010 13:21 UTC www.myspace.com [Source type: General]

.It dismissed 143 applicants because they were carriers, even though none of them had the condition.^ It dismissed 143 applicants because they were carriers, even though none of them had the condition.

^ Even though many experienced an average of 50% reduction in acute pain episodes [ 8 , 9 ], they still had frequent acute pain episodes and high levels of chronic pain.
  • Health and Quality of Life Outcomes | Full text | Hydroxyurea and sickle cell anemia: effect on quality of life 6 February 2010 13:21 UTC www.hqlo.com [Source type: Academic]

^ AMNIOTIC CELLS Use of amniotic cells was the easiest approach to DNA analysis, even though they provide reliable results only after the 17 th week of pregnancy.
  • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]

.It eventually withdrew the requirement, but only after a trainee filed a lawsuit.^ It eventually withdrew the requirement, but only after a trainee filed a lawsuit.

[30]

History

.This collection of clinical findings was unknown until the explanation of the sickle cells in 1904 by the Chicago cardiologist and professor of medicine James B. Herrick (1861-1954), whose intern Ernest Edward Irons (1877-1959) found "peculiar elongated and sickle-shaped" cells in the blood of Walter Clement Noel, a 20-year-old first-year dental student from Grenada, after Noel was admitted to the Chicago Presbyterian Hospital in December 1904 suffering from anaemia.^ "Sickle cell anaemia trait".
  • Sickle Cell Patients Resource - www.sicklecell.md 10 January 2010 1:26 UTC www.sicklecell.md [Source type: Academic]

^ Submissions for the collection: Sickle Cell Disease .
  • Sickle Cell Disease - a knol by Anonymous 10 January 2010 1:26 UTC knol.google.com [Source type: Academic]

^ "Why is Sickle Cell Anaemia only found in Black people?"
  • Sickle Cell Patients Resource - www.sicklecell.md 10 January 2010 1:26 UTC www.sicklecell.md [Source type: Academic]

[31]
Noel was readmitted several times over the next three years for "muscular rheumatism" and "bilious attacks". Noel completed his studies and returned to the capital of Grenada (St. George's) to practice dentistry. .He died of pneumonia in 1916 and is buried in the Catholic cemetery at Sauteurs in the north of Grenada.^ He died of pneumonia in 1916 and is buried in the Catholic cemetery at Sauteurs in the north of Grenada.

[32]
.The disease was named "sickle-cell anaemia" by Vernon Mason in 1922. However, some elements of the disease had been recognized earlier: A paper in the Southern Journal of Medical Pharmacology in 1846 described the absence of a spleen in the autopsy of a runaway slave.^ Sickle cell disease and associated hemoglobinopathies.
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

^ Pulmonary hypertension in sickle cell disease.
  • Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia -- Gladwin and Kato 2005 (1): 51 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

^ Splenic sequestration and sickle cell disease .
  • Sickle Cell Anemia - Kosmix : Reference, Videos, Images, News, Shopping and more... 6 February 2010 13:21 UTC health.kosmix.com [Source type: Academic]

.The African medical literature reported this condition in the 1870s, when it was known locally as ogbanjes ("children who come and go") because of the very high infant mortality rate caused by this condition.^ The African medical literature reported this condition in the 1870s where it was known locally as ogbanjes ("children who come and go") because of the very high infant mortality rate caused by this condition.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ The mortality rate of such infections has been reported to be as high as 10-30%.
  • Sickle Cell Anemia: eMedicine Pediatrics: General Medicine 6 February 2010 13:21 UTC emedicine.medscape.com [Source type: Academic]

^ The African medical literature reported this condition in the 1870s, when it was known locally as ogbanjes ("children who come and go") because of the very high infant mortality rate caused by this condition.

.A history of the condition tracked reports back to 1670 in one Ghanaian family.^ A history of the condition tracked reports back to 1670 in one Ghanaian family.

^ At NIH, acute chest syndrome (ACS) was defined as a patient reported history of one or more episodes of “chest syndrome” or “pneumonia” requiring hospitalization.
  • PLoS ONE: Chronic Hyper-Hemolysis in Sickle Cell Anemia: Association of Vascular Complications and Mortality with Less Frequent Vasoocclusive Pain 6 February 2010 13:21 UTC www.plosone.org [Source type: Academic]

^ The mean follow-up was only three years, and given the documented natural history of the condition one would expect the results to deteriorate with time.
  • Sickle Cell Disease I � Musculoskeletal System 10 January 2010 1:26 UTC www.ptolemy.ca [Source type: Academic]

[33] .Also, the practice of using tar soap to cover blemishes caused by sickle-cell sores was prevalent in the black community.^ Also, the practice of using tar soap to cover blemishes caused by sickle-cell sores was prevalent in the black community.

^ What causes sickle cell disease?
  • Exempla Healthcare Online Library 10 January 2010 1:26 UTC www.exempla.org [Source type: Academic]
  • Sickle Cell Disease, Robert Wood Johnson University Hospital, New Brunswick, NJ, 08903 10 January 2010 1:26 UTC www.rwjuh.edu [Source type: Academic]
  • Sickle Cell Disease 10 January 2010 1:26 UTC www.childrenscentralcal.org [Source type: Academic]
  • Sickle Cell Anemia 6 February 2010 13:21 UTC dwb.unl.edu [Source type: FILTERED WITH BAYES]

^ Also, the practice of using tar soap to cover blemishes caused by sickle-cell sores was prevalent in the African American community.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

[citation needed]
.Linus Pauling and colleagues were the first, in 1949, to demonstrate that sickle-cell disease occurs as a result of an abnormality in the haemoglobin molecule.^ Pulmonary hypertension in sickle cell disease.
  • Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia -- Gladwin and Kato 2005 (1): 51 -- Hematology 6 February 2010 13:21 UTC asheducationbook.hematologylibrary.org [Source type: Academic]

^ Allon M. Renal abnormalities in sickle cell disease.
  • MANAGEMENT AND THERAPY OF SICKLE CELL DISEASE, NIH PUB # 95-2117 10 January 2010 1:26 UTC research.downstate.edu [Source type: Academic]

^ How is sickle cell disease treated?
  • Evansville,IN Sickle Cell Disease; Sickle cell disease changes normal, ... 10 January 2010 1:26 UTC www.stmarys.org [Source type: Academic]

This was the first time a genetic disease was linked to a mutation of a specific protein, a milestone in the history of molecular biology, and it was published in their paper "Sickle Cell Anemia, a Molecular Disease".
.The origin of the mutation that led to the sickle-cell gene was initially thought to be in the Arabian peninsula, spreading to Asia and Africa.^ Edington GM, Lehmann H. Expression of the sickle-cell gene in Africa.
  • Sickle Cell Disease I � Musculoskeletal System 10 January 2010 1:26 UTC www.ptolemy.ca [Source type: Academic]

^ Mortality from sickle cell disease in Africa.
  • NEJM -- Mortality In Sickle Cell Disease -- Life Expectancy and Risk Factors for Early Death 6 February 2010 13:21 UTC content.nejm.org [Source type: Academic]

^ Initiation of prenatal diagnosis of sickle cell disease in Africa.
  • MoonDragon's ObGyn Health Information: Sickle Cell Anemia: Prenatal Diagnosis of Sickle Cell Anemia 6 February 2010 13:21 UTC www.moondragon.org [Source type: Academic]

.It is now known, from evaluation of chromosome structures, that there have been at least four independent mutational events, three in Africa and a fourth in either Saudi Arabia or central India.^ Sickle cell disease is most common in people whose families come from Africa, South or Central America, Caribbean islands, Mediterranean countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia.
  • Hydroxyurea Treatment for Sickle Cell Disease 10 January 2010 1:26 UTC consensus.nih.gov [Source type: Academic]
  • National Institutes of Health Consensus Development Conference Statement: Hydroxyurea Treatment for Sickle Cell Disease — Ann Intern Med 10 January 2010 1:26 UTC www.annals.org [Source type: Academic]

^ It’s most common in people whose families come from Africa, South or Central America (especially Panama), Caribbean islands, Mediterranean countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia.
  • Sickle Cell Anemia, All Sections 15 September 2009 2:51 UTC www.nhlbi.nih.gov [Source type: FILTERED WITH BAYES]

^ It is particularly common among people whose ancestors come from Sub-Saharan Africa, South America, Cuba, Central America, Saudi Arabia, India, and Mediterranean countries such as Turkey, Greece, and Italy.
  • WHO | Genes and human disease 10 January 2010 1:26 UTC www.who.int [Source type: Academic]

.These independent events occurred between 3,000 and 6,000 generations ago, approximately 70-150,000 years.^ These independent events occurred between 3,000 and 6,000 generations ago, approximately 70-150,000 years.
  • Bing Health Article - Sickle-cell disease 10 January 2010 1:26 UTC www.bing.com [Source type: Academic]

^ Stroke, atrophy, and cognitive impairment are major consequences of SCA. Approximately 25% of all patients with SCA will have a neurologic complication over their lifetime; 11% of these complications will occur by age 20 years ( 51 , 52 ).

^ STO occurs in approximately one in 10,000 black babies.
  • SICKLE CELL - A PARENTS GUIDE 6 February 2010 13:21 UTC www.squidoo.com [Source type: General]

[34]

See also

References

  1. ^ Platt OS, Brambilla DJ, Rosse WF, et al. (June 1994). "Mortality in sickle cell disease. Life expectancy and risk factors for early death". N. Engl. J. Med. 330 (23): 1639–44. doi:10.1056/NEJM199406093302303. ISSN 0028-4793. PMID 7993409. http://content.nejm.org/cgi/content/full/330/23/1639. 
  2. ^ Sicklecell.md
  3. ^ Sicklecell.md FAQ: "Why is Sickle Cell Anaemia only found in Black people?
  4. ^ http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_Summary.html
  5. ^ Pearson H (Aug 1977). "Sickle cell anaemia and severe infections due to encapsulated bacteria" (Free full text). J Infect Dis 136 Suppl: S25–30. ISSN 0022-1899. PMID 330779. http://www.nlm.nih.gov/medlineplus/meningitis.html. 
  6. ^ Wong W, Powars D, Chan L, Hiti A, Johnson C, Overturf G (Mar 1992). "Polysaccharide encapsulated bacterial infection in sickle cell anaemia: a thirty year epidemiologic experience" (Free full text). Am J Hematol 39 (3): 176–82. doi:10.1002/ajh.2830390305. ISSN 0361-8609. PMID 1546714. http://www.nlm.nih.gov/medlineplus/sicklecellanemia.html. 
  7. ^ Jadavji T, Prober CG (April 1985). "Dactylitis in a child with sickle cell trait". Can Med Assoc J 132 (7): 814–5. ISSN 0008-4409. PMID 3978504. 
  8. ^ http://www.ejbjs.org/cgi/content/abstract/58/8/1161
  9. ^ "BestBets: How long should an average sickle cell crisis last?". http://www.bestbets.org/bets/bet.php?id=1189. 
  10. ^ "Sickle cell anemia". Mayo Clinic. 2009-04-01. http://www.mayoclinic.com/health/sickle-cell-anemia/DS00324/DSECTION=symptoms. Retrieved 2009-05-02. 
  11. ^ Bandyopadhyay R, Bandyopadhyay SK, Dutta A (Apr 2008). "Sickle cell hepatopathy". Indian J Pathol Microbiol 51 (2): 284–5. doi:10.4103/0377-4929.41698. ISSN 0377-4929. PMID 18603711. http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2008;volume=51;issue=2;spage=284;epage=285;aulast=Bandyopadhyay. 
  12. ^ http://www.wheelessonline.com/ortho/osteomyelitis_infection_in_the_sickle_cell_patient
  13. ^ Smith WR, Penberthy LT, Bovbjerg VE, et al. (Jan 2008). "Daily assessment of pain in adults with sickle cell disease". Ann. Intern. Med. 148 (2): 94–101. ISSN 0003-4819. PMID 18195334. 
  14. ^ Gladwin MT, Sachdev V, Jison ML, et al. (Feb 2004). "Pulmonary hypertension as a risk factor for death in patients with sickle cell disease". N. Engl. J. Med. 350 (9): 886–95. doi:10.1056/NEJMoa035477. ISSN 0028-4793. PMID 14985486. http://content.nejm.org/cgi/content/full/350/9/886. 
  15. ^ Powars DR, Elliott-Mills DD, Chan L, et al. (Oct 1991). "Chronic renal failure in sickle cell disease: risk factors, clinical course, and mortality". Ann. Intern. Med. 115 (8): 614–20. ISSN 0003-4819. PMID 1892333. 
  16. ^ Clarke GM, Higgins TN (Aug 2000). "Laboratory investigation of haemoglobinopathies and thalassemias: review and update". Clin. Chem. 46 (8 Pt 2): 1284–90. ISSN 0009-9147. PMID 10926923. http://www.clinchem.org/cgi/content/full/46/8/1284. 
  17. ^ "BestBets: Does routine urinalysis and chest radiography detect occult bacterial infection in sickle cell patients presenting to the accident and emergency department with painful crisis?". http://www.bestbets.org/bets/bet.php?id=1102. 
  18. ^ http://sickle.bwh.harvard.edu/scd_background.html
  19. ^ http://emedicine.medscape.com/article/778971-overview
  20. ^ Green NS, Fabry ME, Kaptue-Noche L, Nagel RL (Oct 1993). "Senegal haplotype is associated with higher HbF than Benin and Cameroon haplotypes in African children with sickle cell anemia" (Free full text). Am. J. Hematol. 44 (2): 145–6. doi:10.1002/ajh.2830440214. ISSN 0361-8609. PMID 7505527. http://www.nlm.nih.gov/medlineplus/sicklecellanemia.html. 
  21. ^ Kwiatkowski DP (Aug 2005). "How malaria has affected the human genome and what human genetics can teach us about malaria". Am. J. Hum. Genet. 77 (2): 171–92. doi:10.1086/432519. ISSN 0002-9297. PMID 16001361.  Full text at PMC: 1224522
  22. ^ http://www.cdc.gov/ncidod/EID/vol7no6/romi.htm
  23. ^ http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1808464
  24. ^ http://journals.cambridge.org/action/displayAbstract?aid=1361276
  25. ^ Aldrich TK, Nagel RL. (1998). "Pulmonary Complications of Sickle Cell Disease.". in C et al., editors. Pulmonary and Critical Care Medicine (6th ed.). St. Louis: Mosby. pp. pp.1–10. ISBN 0-81511-371-4. 
  26. ^ Charache S, Terrin ML, Moore RD, et al. (May 1995). "Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia". N. Engl. J. Med. 332 (20): 1317–22. doi:10.1056/NEJM199505183322001. ISSN 0028-4793. PMID 7715639. 
  27. ^ Steinberg MH, Barton F, Castro O, et al. (Apr 2003). "Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment". JAMA 289 (13): 1645–51. doi:10.1001/jama.289.13.1645. ISSN 0098-7484. PMID 12672732. http://jama.ama-assn.org/cgi/content/full/289/13/1645. 
  28. ^ Platt OS (Mar 2008). "Hydroxyurea for the treatment of sickle cell anemia". N. Engl. J. Med. 358 (13): 1362–9. doi:10.1056/NEJMct0708272. ISSN 0028-4793. PMID 18367739. 
  29. ^ Walters MC, Patience M, Leisenring W, et al. (August 1996). "Bone marrow transplantation for sickle cell disease". N. Engl. J. Med. 335 (6): 369–76. doi:10.1056/NEJM199608083350601. ISSN 0028-4793. PMID 8663884. http://content.nejm.org/cgi/pmidlookup?view=short&pmid=8663884&promo=ONFLNS19. 
  30. ^ Anonymous (4 January 1981). "Air force academy sued over sickle cell policy". New York Times. http://query.nytimes.com/gst/fullpage.html?sec=health&res=9807EFD7163BF937A35752C0A967948260. Retrieved 21 December 2008. 
  31. ^ Herrick, J.B. (1910). "Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia". Archives of Internal Medicine 6: 517–521. 
  32. ^ Savitt TL, Goldberg MF (Jan 1989). "Herrick's 1910 case report of sickle cell anemia. The rest of the story" (Free full text). JAMA 261 (2): 266–71. doi:10.1001/jama.261.2.266. ISSN 0098-7484. PMID 2642320. http://www.nlm.nih.gov/medlineplus/sicklecellanemia.html. 
  33. ^ Konotey-Ahulu FID. Effect of environment on sickle cell disease in West Africa: epidemiologic and clinical considerations. In: Sickle Cell Disease, Diagnosis, Management, Education and Research. Abramson H, Bertles JF, Wethers DL, eds. CV Mosby Co, St. Louis. 1973; 20; cited in Desai, D. V.; Hiren Dhanani (2004). "Sickle Cell Disease: History And Origin". The Internet Journal of Haematology 1 (2). ISSN 1540-2649. http://www.ispub.com/ostia/index.php?xmlFilePath=journals/ijhe/vol1n2/sickle.xml. 
  34. ^ Desai, D. V.; Hiren Dhanani (2004). "Sickle Cell Disease: History And Origin". The Internet Journal of Haematology 1 (2). ISSN 1540-2649. http://www.ispub.com/ostia/index.php?xmlFilePath=journals/ijhe/vol1n2/sickle.xml. 

External links


Simple English

Sickle cell anemia is a disease passed down from family to family that affects red blood cells. It changes the cells from flexible circles into rigid crescents. This creates clotting in veins and can cause a heart attack. Drepanocytes has the same meaning but in a more scientific way. This disease is usually created in child hood. This disease is a life long disease and a life long blood disorder, sickle cell disease or sickle-cell anaemia (or drepanocytosis), characterized by red blood cells that assume an abnormal, rigid, sickle shape. Sickling decreases the cells' flexibility and results in a risk of various complications. The sickling occurs because of a mutation in the hemoglobin gene. Life expectancy is shortened, with studies reporting an average life expectancy of 42 and 48 years for males and females, respectively.[1]

Sickle-cell disease, usually presenting in childhood, occurs more commonly in people (or their descendants) from parts of tropical and sub-tropical regions where malaria is or was common. One-third of all aboriginal inhabitants of Sub-Saharan Africa carry the gene[2], because in areas where malaria is common, there is a survival value in carrying only a single sickle-cell gene (sickle cell trait).[3] Those with only one of the two alleles of the sickle-cell disease are more resistant to malaria, since the infestation of the malaria plasmodium is halted by the sickling of the cells which it infests.


Citable sentences

Up to date as of December 20, 2010

Here are sentences from other pages on Sickle-cell disease, which are similar to those in the above article.








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