βS-gene-cluster haplotypes in sickle cell anemia : clinical implications

摘要: Restriction endonuclease analysis was used to detect alpha-gene deletions and determine the haplotypes in DNA of beta S-gene-cluster [Benin, Central African Republic (CAR), Senegal] 221 patients with sickle cell anemia (SS). The clinical expression SS modified by polymorphisms status (alpha-thalassemia-2). overall risk soft tissue organ failure caused obliterative vasculopathy (including stroke, renal failure, chronic lung disease cor pulmonale, leg ulcers, young adult death) increased threefold those a CAR haplotype decreased Senegalese chromosome (p = 0.003). In presence haplotype, patient's health is better, it always worse. With Benin, intermediate. Acute recurrent events including hospitalized crisis, bone infarction, infection are frequency haplotype. most acute chest syndrome equivalent Benin or haplotypes. United States, alpha-thalassemia-2 co-inherited randomly among occurring during childhood minimally effected this co-inheritance. decreased. After age 20 years, painful episodes lumbar dorsal area who had association degenerative disease.(ABSTRACT TRUNCATED AT 250 WORDS)