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 populati...

a Thalassemia modifies the hematologic expression of homozygous sickle cell (55) disease, resulting in increased total hemoglobin and HbA2 and decreased HbF. mean cell volume, reticulocytes. irreversibly sickled cells. and bilirubin levels. The age at which these changes develop in children with 55 disease is unknown. Ascertainment of globin gene status in a large representative sample of child...

objective:  the aim of this study was to assess clinical laboratory funding for differential diagnosis of sickle cell disease (scd) and other associated disorders for better understanding of clinical types and prevention of sickling events. material and methods:  this is a descriptive crossed-sectional study that analyzed the peripheral blood film, sickle cell preparation, hemoglobin electroph...

potent induction of fetal hemoglobin (hbf) production results in alleviating the complications of β-thalassemia and sickle cell disease (scd). hbf inducer agents can trigger several molecular signaling pathways critical for erythropoiesis. janus kinase/signal transducer and activator of transcription (jak/stat), mitogen activated protein kinas (mapk) and phosphoinositide 3-kinase (pi3k) are con...

Epiretinal membranes at the macula were seen in 4% of the eyes of 355 patients with homozygous sickle cell (SS) disease and sickle cell haemoglobin-C (SC) disease under the age of 60 years. The presence of proliferative sickle retinopathy (PSR), the extent of involvement of PSR, and vitreous haemorrhage all constitute risk factors for the formation of epiretinal membranes. The occlusion of PSR ...

The term sickle cell disease embraces a group of genetic conditions in which pathology results from the inheritance of the sickle cell gene either homozygously or as a double heterozygote with another interacting gene. The spectrum of resulting conditions is therefore influenced by the geography of individual hemoglobin genes, but in most populations, the commonest genotype at birth is homozygo...

BACKGROUND Sickle cell disease (SCD) is a class of hemoglobinopathy resulting from a single mutation in the ß-globin chain inducing the substitution of valine for glutamic acid at the sixth amino acid position which leads to the production of abnormal haemoglobin (haemoglobin S [HbS]). Studies demonstrated the implication of oxidative stress in the development of the sickle cell disease. METH...

Objective(s) Sickle cell disease is a genetic disorder characterized by chronic haemolytic anaemia. Haemoglobin S containing red blood cells may be susceptible to oxidative stress due to imbalance between production of reactive oxygen species and the countering effect of the various antioxidants present in the body. Materials and Methods We evaluated some antioxidant enzymes which include gl...

Homozygous alpha-thalassemia has the beneficial effect in sickle cell anemia of reducing the hemolytic severity while changing several other hematological parameters. We examined in detail the cellular basis of some of these hematologic alterations. We find that the broad distribution in erythrocyte density and the large proportion of dense cells associated with sickle cell anemia are both redu...