Alpha thalassaemia and the macular vasculature in homozygous sickle cell disease.

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Alpha thalassaemia and the macular vasculature in homozygous sickle cell disease.

The interaction of homozygous alpha thalassaemia 2 with homozygous sickle cell (SS) disease results in a generally milder haematological picture with less intravascular sickling, less haemolysis, and higher haemoglobin levels. Clinically, patients are generally more mildly affected, though not all vaso-occlusive complications are reduced. Thus there is a possibility that the advantages gained b...

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Macular vasculature, visual acuity, and irreversibly sickled cells in homozygous sickle cell disease.

Observations of visual acuity and the conjunctival, macular, and perimacular vascularity have been assessed in patients with homozygous sickle cell (SS) disease. There were 17 matched pairs, each consisting of one patient with a high count (greater than or equal to 15%) and one with a low count (less than or equal to 5%) of irreversibly sickled cells (ISCs). The macular vascular bed was assesse...

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Influence of alpha thalassaemia on the retinopathy of homozygous sickle cell disease.

Homozygous alpha+ thalassaemia (alpha-/alpha-) ameliorates some of the clinical manifestations of homozygous sickle cell (SS) disease but its effect on retinal complications remains unknown. This has been assessed by visual examination and fluorescein angiography in 39 subjects with SS disease and homozygous alpha+ thalassaemia and in 39 age/sex matched controls with SS disease but with a norma...

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Steady state hemoglobin concentration and packed cell volume in homozygous sickle cell disease patients in Lagos, Nigeria

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...

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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...

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ژورنال

عنوان ژورنال: British Journal of Ophthalmology

سال: 1983

ISSN: 0007-1161

DOI: 10.1136/bjo.67.11.779