Sickle cell disease and other hemoglobinopathies.
نویسنده
چکیده
The term sickle cell disease (SCD) (OMIM database No. 603903) encompasses a group of genetic disorders characterized by chronic hemolysis and intermittent episodes of vascular occlusion that cause recurrent episodes of severe pain and a wide variety of other disease manifestations. Specialized comprehensive medical care markedly reduces mortality in infancy and early childhood by preventing some disease-related complications and limiting the severity and sequelae of others.
منابع مشابه
Assessing clinical laboratory funding of Sickle Cell Disease and others associated Disorders in Khuzestan Province
Objective: The aim of this study was to assess clinical laboratory funding for differential diagnosis of sickle cell disease (SCD) and other associated disorders for better understanding of clinical types and prevention of sickling events. Material and Methods: This is a descriptive crossed-sectional study that analyzed the peripheral blood film, sickle cell preparation, hemoglobin electroph...
متن کاملGenotyping of Individuals with Hemoglobinopathies in Beja Tribes and Other Minor Groups in Port Sudan, Eastern Sudan
ABSTRACT Background and objectives: This study aimed to characterize the spectrum of β-thalassemia mutations and haplotypes of sickle cell anemia in Beja tribes and other minor groups living in Port Sudan, Sudan. Methods: This descriptive cross-sectional study was carried out from March 2011 to July 2013. Overall, 209 anemic patients were screened for hemoglobinopathy ...
متن کاملElucidation of βs/ Globin Gene clusters Haplotypes Related to Sickle Cell Anemia in Khuzestan Province, Southwest of Iran
Background & objectives: The researcher clarified that β/Globin gene cluster haplotypes in patients with sickle cell anemia provide useful population data as predictors of the disease severity, gene flow, and the origins of sickle cell mutation in this region. Materials and methods: A total of 150 subjects was investigated in two different groups for five polymorphism restriction site...
متن کاملSickle cell disease and related hemoglobinopathies.
Objectives 1. Describe different combinations of normal and abnormal hemoglobins and their phenotypic expression 2. Describe the pathophysiology of vasooclusion secondary to hemoglobinopathies 3. Define and describe hand-foot syndrome 4. Describe features of osteomyelitis secondary to sickle cell disease and its differentiation from bone infarct 5. Describe features of avascular necrosis of bon...
متن کاملتظاهرات اسکلتی- عضلانی پیشرفته در یک بیمار مسن مبتلا به بیماری سلول داسی شکل، یک مورد نادر
Sickle cell disease is the most common type of hemoglobinopathies in the world that is caused by abnormal beta globin chain in hemoglobin. The disease is usually diagnosed in the first decade of life. Bone involvement is one of the most common clinical manifestations both in the acute setting (painful vaso-occlusive crises), and/or as a source of chronic disability (such as avascular necrosis)....
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ورودعنوان ژورنال:
- International anesthesiology clinics
دوره 42 3 شماره
صفحات -
تاریخ انتشار 2004