Sickle cell disease is characterized by a very heterogeneous clinical course among patients with the same mutations for sickle hemoglobin (HbS). anemia (SCA) hereditary hemoglobinopathy caused homozygosity of point mutation in beta-globin gene, which leads to substitution glutamic acid valine sixth position.

CNTO 530 is an erythropoietin receptor agonist MIMETIBODYTM construct. CNTO 530 has been shown to be active in a number of rodent models of acquired anemia (e.g. renal insufficiency and chemotherapy induced anemia). We investigated the efficacy of CNTO 530 in murine models of β-thalassemia and sickle cell anemia (Berkeley mice). β- thalassemic mice are deficient in expression of α-globin chain ...

who had been on continuous opiate infusion since September, 1996. Far from the consultants taking umbrage because I pointed out that the patient could not have been in sickle crisis for 6 months, they were happy to discuss with me the way forward. To put a false dichotomy between NHS practice and private practice as Bevan does can work only to the detriment of the patient. NHS consultants and I...

The Cooperative Study of Sickle Cell Disease reported that dactylitis, severe anemia, and leukocytosis in very young children with sickle cell disease (SCD) increased the risk of later adverse outcomes, including death, stroke, frequent pain, and recurrent acute chest syndrome. This model has not been validated in other cohorts. We evaluated its performance in the Dallas Newborn Cohort, a newbo...

Sickle cell disease (SCD) is an autosomal recessive inheritance disorder that affects the beta-globin gene and results in the replacement of the amino acid glutamic acid by valine in the β chain of the hemoglobin molecule, producing erythrocytes with defective forms and functions. A range of pathological conditions is associated with SCD, however, one in particular stands out by gravity. Acute ...

Background: Molecular genetic factors regulating hemoglobin F (Hb F) expression are important modifiers of the severity of sickle cell anemia (SS). Methods: The prevalence of XmnI polymorphic site, the Gg:Ag ratio and the Hb F level were determined using PCR-RFLP procedure, HPLC and alkaline denaturation method, respectively, in various haplotypes of 52 patients with SS, 18 patients with sickle...

Objectives.  The probability of success with spinal cord stimulation (SCS) depends largely on appropriate patient selection. Here, we have assessed the predictive value of pain etiology as it relates to pain relief with SCS as part of a prospective multicenter clinical trial. Methods.  Sixty-five subjects with chronic and intractable pain tested an epidural SCS system. Subjects reported pain ra...

Creatine kinase activity was measured in 28 patients in the steady state of sickle cell disease and ranged from 4-45 IU/l, comparable with that found in healthy adult caucasians. Creatine kinase activity was also measured in 14 patients admitted consecutively for the treatment of vaso-occlusive sickle cell crises. Creatine kinase activity remained within the normal range in eight of these 14 pa...

The Cooperative Study of Sickle Cell Disease reported that dactylitis, severe anemia, and leukocytosis in very young children with sickle cell disease (SCD) increased the risk of later adverse outcomes, including death, stroke, frequent pain, and recurrent acute chest syndrome. This model has not been validated in other cohorts. We evaluated its performance in the Dallas Newborn Cohort, a newbo...

Subclinical Cushing's syndrome (SCS) is a condition of slight but chronic cortisol excess in patients with adrenal incidentaloma (AI) without typical signs and symptoms of Cushing's syndrome. Adiponectin has potent roles in modulating energy balance and metabolic homeostasis and acts in opposition to glucocorticoids. This study aimed to evaluate adiponectin level in SCS and nonfunctional AI (NA...