Background: Sickle cell disease is a genetic disorder of hemoglobin causing myriad of pathology including anemia. The purpose of this study was to evaluate the baseline values of steady state hemoglobin and packed cell volume as a guide to managing the early recognition of hemolytic crises in sickle cell anemia. Methods: A cross-sectional study was conducted among the sickle cell patients atte...

Background: Sickle cell disease occurs due to a mutation in β chains and the substitution of valine instead of glutamate in the sixth position of the ß-chain that causes polymerization and vascular blockage. The aim of this study was to compare the serum C, S proteins and factor V Leiden between sickle cell patients and the control group. Materials and Methods: In this case-control study, perf...

a case of sickle cell hb d disease is reported in  young iranian male. the father of whom carried an as sickle cell trait and the mother an ad trait. this disease was diagnosed by hb electrophoresis. agar gel electrophoresis sickling and solubility tests.this is the first case of sickle cell hb d disease reported in iran.

OBJECTIVE To investigate whether attacks of acute chest syndrome affected pulmonary artery pressure in patients homozygous for sickle cell disease. MAIN OUTCOME MEASURES Pulmonary artery pressure, assessed by non-invasive echocardiographic techniques. PATIENTS 20 patients with homozygous sickle cell disease with a history of at least six episodes of acute chest syndrome and in 20 age, sex, ...

In sickle cell disease transfusions improve blood flow by reducing the proportion of red cells capable of forming sickle hemoglobin polymer. This limits hemolysis and the endothelial damage that result from high proportions of sickle polymer-containing red cells. Additionally, transfusions are used to increase blood oxygen carrying capacity in sickle cell patients with severe chronic anemia or ...

Sickel cell disease is common in urban areas of Britain and it is estimated that in London alone there are nearly 2000 patients. One hundred and eighty four patients with sickle cell disease are known to the Central Middlesex Hospital, and 155 of those attend the sickle cell clinic regularly. The commonest cause for admission to hospital is acute painful or vaso-occlusive crisis, which accounts...

Hepatic dysfunction is a frequent manifestation in patients with sickle cell anemia. It is usually a multifactorial process. A rare benign form of extreme hyperbilirubinemia, presumably due to intrahepatic sickling, may be the cause. We report a 9 year old girl with sickle-thalassemia hemoglo binopathy, presenting with profound jaundice. Sickle cell disease is often mild in the Iranian popu...

sickle cell disease (scd) is a known inherited hemoglobin disorder featured by the presence of sickle shaped erythrocytes in the blood. it can cause cerebrovascular accident (cva) in adults and children and is responsible for the majority of the strokes in children. repeated blood transfusion are often required in an attempt to dilute blood thus reducing the risk of vaso-occlusion and stroke in...

sickle cell disease is a genetic haemoglobinopathy with consequent haemolysis and anaemia. it is of interest to study its effect on red cell indices beside haemoglobin concentration.the objective of the study is to determine the values of red cell indices in preschool-age children with sickle cell anaemia.we conducted a cross-sectional study including 97 children with sickle cell anaemia aged s...

background: the vascular response to recurrent tissue hypoxia and reperfusion following red blood cell sickling causes acute chest syndrome and chronic lung disease. the purpose of this study was to determine the pattern of chronic lung lesions and possible risk factors in sickle cell patients in lagos, nigeria.methods: from july 2012 to april 2013, pulmonary function test (pft) and chest-x-ray...