Background: The integrity of the autonomic nervous system (ANS) is essential for keeping physiological processes stable, even under stress. Since there growing interest in heart rate variability (HRV) analysis noninvasive assessment ANS sickle cell anemia (SCA) patients, we studied behavior presence a stressor that simulates daily-life multitasking, Glittre ADL test (GA-T). Objectives: To evalu...

Pressor and heart rate changes following change in posture without or with L-arginine supplementation (1g/day for 6 weeks) were studied in 28 sickle cell anemia (SCA) and 32 non-sickle cell anemia (NSCA) subjects. Change in posture increased HR (p<0.01), RPP (p<0.05) in both groups of subjects, MABP (p<0.05) in SCAS but reduced MABP (p<0.01) in NSCAS and PP (p<0.01) in SCAS. L-Ar...

OBJECTIVE To compare the pregnancy and neonatal outcome in sickle cell disease and trait women. METHOD The retrospective comparative study was conducted at Abha General Hospital, Abha, Saudi Arabia, from January 2009 to December 2011. The records of women having sickle cell disease and trait were reviewed for antenatal and postnatal complications. Pregnancy and foetal outcome was also compare...

INTRODUCTION This is the first published report of a young girl with co-inherited sickle cell-β+ thalassemia and cystic fibrosis. Although a small subset of patients with co-inherited cystic fibrosis and other hemoglobinopathies have been reported, this patient developed early hematologic and pulmonary complications that were more severe than the previous cases. To assess pulmonary co-morbiditi...

Polymers of deoxyhemoglobin S deform sickle cell anemia red blood cells into sickle shapes, leading to the formation of dense, dehydrated red blood cells with a markedly shortened life-span. Nearly four decades of intense research in many laboratories has led to a mechanistic understanding of the complex events leading from sickling-induced permeabilization of the red cell membrane to small cat...

Several episodes of acute hepatic enlargement associated with a dramatic fall in haemoglobin concentration were observed in two patients with sickle cell anaemia. No appreciable disturbances of liver function or signs of cardiac failure were evident. The most likely mechanism was sequestration of sickled erythrocytes in the liver. This complication, which may have a basis similar to that of spl...

We present the findings in a patient having sickle cell disease who developed multilobar pneumonia. Cultures of bronchial aspirates and histologic specimens grew Cryptococcus neoformans. There was neither spontaneous clearing of the infection nor a response to bactericidal antibiotics. The patient had no underlying malignant neoplasm or immunodeficiency as indicated by history, physical examina...

Introduction. Hypertransfusion refers to chronic blood transfusion therapy aimed at ameliorating disease complications in various haemopathies particularly the haemoglobinopathies. In sickle cell disease, hypertransfusion is aimed at maintaining patient's haemoglobin level at 10 to 11 g/dL using haemoglobin AA blood and its resultant dilutional effect on sickle haemoglobin is sustained by inter...

Although the primary origin of sickle cell disease is a hemoglobin disorder, many types of cells contribute considerably to the pathophysiology of the disease. The adhesion of neutrophils to activated endothelium is critical in the pathophysiology of sickle cell disease and targeting neutrophils and their interactions with endothelium represents an important opportunity for the development of n...