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Sickle-cell disease

The Lancet, 2004
With the global scope of sickle-cell disease, knowledge of the countless clinical presentations and treatment of this disorder need to be familiar to generalists, haematologists, internists, and paediatricians alike. Additionally, an underlying grasp of sickle-cell pathophysiology, which has rapidly accrued new knowledge in areas related to erythrocyte
Marie J, Stuart, Ronald L, Nagel
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Sickle Cell Disease

Archives of Pediatrics & Adolescent Medicine, 1979
Sir.—A recentJournalreport by Buchanan and Glader entitled, "Leukocyte Counts in Children With Sickle Cell Disease" (132:396-398, 1978), concluded in part that "an absolute band count greater than 1,000/μL suggests bacterial infection in children with sickle cell anemia." They found such a level in 14 of 16 episodes of bacterial infection but in only ...
C, Pegelow, D, Powars, G D, Overturf
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Sickle cell disease

Pediatric Clinics of North America, 2002
Sickle cell disease is a serious and life threatening disease that affects approximately 1 in 600 African-Americans. Since its first description in 1910 by Herrick, our understanding and treatment of this disease has grown tremendously. Most pediatricians will encounter one or more children with sickle cell disease and should have a thorough ...
Jason, Fixler, Lori, Styles
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Sickle Cell Disease

Hematology/Oncology Clinics of North America, 2005
The sickling disorders are a group of inherited diseases of the hemoglobin molecule characterized by chronic hemolytic anemia, increased susceptibility to infection, intermittent acute ‘crises,’ and progressive organ dysfunction as a result of vasoocclusion by the sickle erythrocytes.
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SICKLE CELL DISEASE

Pediatric Clinics of North America, 1996
The identification of genetic mutation that causes sickle cell disease 35 years ago has not yet led to a widely applicable, specific therapy that corrects the underlying abnormality of hemoglobin. Nevertheless, recent progress in understanding the pathophysiology and natural history of sickling disorders has led directly to important prophylactic and ...
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Sickle cell disease

2015
People with sickle cell disease, a chronic hemolytic anemia, present with a wide variety of neurological syndromes, including ischemic and hemorrhagic stroke, anterior and posterior territory transient ischemic attacks (TIAs), "soft neurological signs," seizures, headache, coma, visual loss, and altered mental status.
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Sickle Cell Eye Disease

Archives of Ophthalmology, 1985
To the Editor. —The article by Garty et al 1 in the OctoberArchivespresented the case reports of two children in whom sickle cell vaso-occlusive crisis was associated with periorbital swelling and frontal and orbital bone infarction. The relative infrequency of these findings in patients with sickle cell makes the article an important addition to our ...
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Management of sickle cell disease

BMJ, 2008
Hypoxia, hemolysis and infection are more or less associated in patients affected with sickle cell disease. Treatment is based on a programme including regular lifestyle, hydration, folic acid supply, prevention of pneumococcal infections and cerebrovascular events in children, regular follow-up in specialised centres allowing precocious screening and ...
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Fetal Hemoglobin, Sickling, and Sickle Cell Disease

Advances in Pediatrics, 1990
Increased numbers of F cells and large amounts of Hb F/F cell appear to produce clinical benefit in rare variants of sickle cell disease and probably in more commonly encountered patients. Fetal hemoglobin interferes with polymerization of Hb S in vitro, but laboratory studies carried out with homogeneous hemoglobin solutions are inadequate models of ...
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Sickle Cell Disease

AJN, American Journal of Nursing, 2023
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