Micro- and macrovascular function in children with sickle cell anaemia and sickle cell haemoglobin C disease
نویسندگان
چکیده
منابع مشابه
Sickle-cell anaemia, sickle-cell thalassaemia, sickle-cell haemoglobin C disease, and asymptomatic haemoglobin C thalassaemia in one Ghanaian family.
A Ghanaian family is described in which a sickle-cell haemoglobin C man married to a sickle-cell thalassaemia woman produced 12 children (eight alive). Four children have sickle-cell anaemia, two sickle-cell haemoglobin C disease, one has sickle-cell thalassaemia, and one is asymptomatic haemoglobin C thalassaemia.It is emphasized that the contribution that adult sickle-cell disease patients ma...
متن کاملThe pathology of sickle cell haemoglobin C disease and sickle cell anaemia.
The purpose of this communication is to describe the necropsy findings in three patients dying of sickle cell haemoglobin C disease and two of sickle cell anaemia. In southern Ghana there is a high incidence of abnormal haemoglobins in the population, the incidence of haemoglobin S being 18% and of haemoglobin C 12% (Edington and Lehmann, 1954a). Haemoglobin G has also been described (Edington ...
متن کاملSickle Cell Disease and Sickle Cell Anaemia
Sickle cell haemoglobin (HbS) results from an autosomal recessively inherited mutation in which the 17th nucleotide of the beta globin gene is changed from thymine to adenine and the amino acid glutamic acid is replaced by valine at position 6 in the beta globin chain. [1, 2]Sickle cells have a reduced deformability and are easily destroyed, causing occlusion of the microcirculation and a chron...
متن کاملSickle-cell haemoglobin C disease in London.
The manifestations of the sickling disorders are becoming increasingly familiar to clinicians in Great Britain. One of these disorders, sickle-cell haemoglobin disease, has hitherto received little attention, being regarded as a relatively mild condition. This paper describes some of the distinctive clinical features of the disease as seen in a series of nine cases which have recently presented...
متن کاملEffect of Age on Blood Rheology in Sickle Cell Anaemia and Sickle Cell Haemoglobin C Disease: A Cross-Sectional Study
OBJECTIVES Blood rheology plays a key role in the pathophysiology of sickle cell anaemia (SS) and sickle cell haemoglobin C disease (SC), but its evolution over the lifespan is unknown. MATERIALS AND METHODS Blood viscosity, red blood cell (RBC) deformability and aggregation, foetal haemoglobin (HbF) and haematocrit were measured in 114 healthy individuals (AA), 267 SS (161 children + 106 adu...
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ژورنال
عنوان ژورنال: Blood Cells, Molecules, and Diseases
سال: 2017
ISSN: 1079-9796
DOI: 10.1016/j.bcmd.2017.02.001