Studies of splenic function were carried out on patients with sickle-cell diseases by the measurement of the clearance of autologous heat-damaged 99mTc-labelled erythrocytes from circulation and into the spleen, the spleen area by a scintillation scanning, the enumeration of pitted erythrocytes by direct-interference microscopy, and the percentage of irreversibly sickled cells (ISC) and of cell...

RATIONALE Pulmonary complications of sickle cell anemia (Hb-SS) commonly cause morbidity, yet few large studies of pulmonary function tests (PFTs) in this population have been reported. OBJECTIVES PFTs (spirometry, lung volumes, and diffusion capacity for carbon monoxide [DLCO]) from 310 adults with Hb-SS were analyzed to determine the pattern of pulmonary dysfunction and their association wi...

Low steady state haemoglobin oxygen saturation in patients with sickle cell anaemia has been associated with the degree of anaemia and haemolysis. How much pulmonary dysfunction contributes to low saturation is not clear. In a prospective study of children and adolescents with sickle cell disease aged 3-20 years at steady state and matched controls, 52% of 391 patients versus 24% of 63 controls...

PURPOSE OF REVIEW Recent developments in the diagnosis and management of sickle cell ocular manifestations are reviewed to enable the clinician to better manage the ophthalmic care of these patients. RECENT FINDINGS Research over the past year has focused upon systemic and ocular clues to the presence of sickle cell retinopathy. In addition, newer imaging modalities, such as spectral domain o...

Sickle cell disease is a genetic hemoglobinopathy in which a significant number of red blood cells carry hemoglobin-S as opposed to normal red blood cells that contain hemoglobin-A. Under certain conditions such as hypoxia, acidosis, and hypothermia, the red blood cells containing hemoglobin-S will sickle, leading to occlusion of the microvasculature. As such, patients with sickle cell disease ...

Hemodynamic studies in 13 unselected patients with sickle cell anemia are reported. Twelve of these patients were also studied during mild exercise. The cardiac output at rest was elevated in all and rose significantly with exercise in 9 of the 12 patients. The importance of the increased percentage extraction of oxygen by the tissues in modifying the response of the cardiac output is discussed...

This communication describes a very simple, rapid and inexpensive point-of-care diagnostic test for sickle cell disease (SCD) that can conclusively differentiate between blood samples from normal healthy individuals, sickle cell trait carriers and SCD patients using the characteristic blood stain patterns produced by each sample on paper.

The presence of hypersplenism and or respond to intravenous particulate functional asplenia occurring concomiantigen, retains the splenic reservoir tantly in a child with sickle cell anemia function to pool platelets. This reserprompted a study of the splenic platevoir function is lost in the older patient let reservoir in this hemoglobinopathy. in whom the spleen has become autoThe young child...

BACKGROUND Haptoglobin genotypes, and interleukin-6 and -8 participate in the pathophysiology of sickle cell anemia. The expression of cytokines is regulated by genetic mechanisms however the effect of haptoglobin polymorphisms on these cytokines is not fully understood. This study aimed to compare the frequency of haptoglobin genotypes and the interleukin-6 and -8 concentrations in sickle cell...