The roles of genetic and non-genetic factors in the haematology, growth and clinical features of sickle cell disease have been studied in nine identical twin pairs (six homozygous sickle cell disease, three sickle cell-haemoglobin C disease). A comparison group of 350 age-gender matched sibling pairs, selected to have an age difference of <5 years, was used for assessing the concordance of nume...

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...

During a routine study of the parents of a 3month-old Jamaican child with the sickle-cell trait the father was found to have a haemoglobin electrophoretic pattern identical to that of homozygous sickle-cell disease. Because he was perfectly well and had no haematological abnormalities detailed studies of his haemoglobin constitution were undertaken. These indicate that he is heterozygous for bo...

Sickle Cell Disease (SCD) is a structural haemoglobinopathy which is extremely diverse in its presentation regarding disease severity and organ involved. The homozygous form if poorly managed gives rise to numerous life threatening conditions which are otherwise avoidable. Here we report the case of a male adolescent with homozygous SCD who presented with haemolytic anaemia, massive ascites, he...

BACKGROUND Sickle cell disease, the most common hereditary blood disease in the world, is the result of an atypical hemoglobin called S (Hb S) which, when homozygous (Hb SS) is the cause of sickle cell anemia. Changes of puberty, correlated with a delayed growth spurt, begin late in both male and female sickle cell anemia individuals with repercussions on sexuality and reproduction. The objecti...

OBJECTIVE Hemochromatosis is an autosomal recessive disease that is one of the most important reasons for iron overload. Sickle cell disease is a hemoglobinopathy that occurs as a result of a homozygous mutation in the hemoglobin gene. Erythrocyte transfusion is frequently used in the treatment of this disease. Iron overload as a result of transfusion is important in the mortality and morbidity...

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...

Angioid streaks were observed in 21 of 242 patients with homozygous sickle cell disease. Two morphological types were observed. There is no evidence that angioid streaks in Jamaican patients are related to pseudoxanthoma elasticum.

There are about 50,000 people in the United States who are homozygous for the sickle hemoglobin gene and thus have sickle cell anemia. Sickle cell anemia is primarily seen in persons of African heritage, about 1 in 14 of whom is an asymptomatic carrier (a heterozygote). One in 700 newborns of African heritage is affected.1 Although it is the most severe of the common sickle cell diseases (which...