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...

Sickle cell intrahepatic cholestasis (SCIC) is an uncommon but severe complication in sickle cell disease (SCD) patients homozygous for hemoglobin (Hb) S or with Hb S/ thalassemia.1–3 It is clinically characterized by marked conjugated hyperbilirubinemia, right upper quadrant pain, enlarged liver and moderately elevated hepatic enzymes. In more severe cases, coagulopathy and renal dysfunction m...

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...

Although sickling and solubility tests and peripheral blood film methods are today available for sickle cell disease screening in Uganda, their reliability and ease of applicability have not been determined. This study was therefore carried out to determine the reliability of sickling and solubility tests and peripheral blood film method for screening for SCD in Uganda. This was descriptive lab...

An increase in hydrogen ion dissociation was found on sickling due to sickle cell haemoglobin polymerization. Since a decrease in pH favours sickling, this might enhance the sickling process in a vicious cycle.

Cyanate and 2,3-diphosphoglycerate (2,3-DPG) both influence the oxygen affinity of hemoglobin. The studies presented here concern the effects of these compounds on the sickling phenomenon. The inhibitory effect of cyanate on sickling is largely due to the fact that it increases the percentage of oxyhemoglobin S at a given oxygen tension. In addition, cyanate inhibits sickling by a mechanism tha...

BACKGROUND Several plant extracts from Rutaceae family are currently used to the management of sickle cell disorder (SCD) in the African. Few reports have shown that extracts from Zanthoxyllum or Fagara genus demonstrated anti-sickling property. This study investigates the in vitro antisickling and antioxidant properties of extracts from Zanthoxyllum heitzii. METHODS The sickling of red blood...

Knowledge of the detailed molecular mechanisms of the sickling process has led to an understanding of factors precipitating crises and hence allowed a reasoned approach to therapy. At present amelioration of the symptoms of sickle cell disease rests primarily on the avoidance and prompt treatment of the basic precipitating factors of the sickling process--fever, hypoxia, acidosis, and dehydrati...

Neuraminic (sialic) acid concentrations in serum from normal and sickle cell (HbSS) subjects were determined for discrete age groups from childhood through adolescence. Values in sickle cell disease were consistently lower over the entire age range. We further investigated the effect of exogenous sialic acid on the rate of sickling reversion of HbSS erythrocytes and demonstrated that this compo...