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...

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 atten...

background: sickle cell disease (hbss) is a major health problem in nigeria and ma-laria has been implicated as a leading cause of morbidity/mortality in sickle cell disease patients. few reasons were put forward to explain the observed morbidity/mortality of hbss subjects due to plasmodium falciparum (p. falciparum) malaria. objectives: to determine the level of immunoglobulin classes (igm, ig...

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.

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: children affected with sickle cell disease (scd) are at increased risk for severe morbidity and mortality, especially during the first 3-5 years of life. it is suggested that early treatment can improve the condition. the aim of this pilot study was to estimate the incidence of hemoglobin s (hbs) by umbilical cord blood screening in khorramshahr and abadan cities in southwest of ira...