Background: The inherited disorders of haemoglobin are the commonest single gene disorder of the world population. Sickle cell anaemia being the most widespread and numerically the most important haemoglobinopathy in the world today. The major features of sickle cell disease (SCD) in most patients are lifelong anaemia and the consequences of recurrent vaso-occlusion. Many complications of SCD i...

BACKGROUND Genes for haemoglobin S are found in high frequencies in Nigeria. However, there is little information on beta thalassemia in sickle cell anaemia in this population. The clinical presentation of HbS- β thalassemia is enormously variable, ranging from an asymptomatic state to a severe disorder similar to homozygous sickle cell disease. MATERIALS AND METHODS Haemoglobin A2 and HbF we...

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 ...

An automated technique is described which is capable of detecting sickle-cell haemoglobin and differentiating the sickle-cell trait from sickle-cell anaemia. The method is based upon the Itano solubility test and utilizes Technicon equipment.

The majority of patients with sickle-cell anaemia live in the underdeveloped nations where endemic parasitic diseases are prevalent and this may exacerbate the severity of steady-state anaemia in infected patients. We studied the impact of intestinal parasites on haematological parameters of sickle-cell anaemia patients aged 18-35 years in Kano, Nigeria. Of 100 patients studied, 27 were found t...

BACKGROUND Sickle cell anaemia (SCA) is associated with structural manifestations in the hepatobiliary axis. This study aimed to investigate the hepatobiliary ultrasonographic abnormalities in adult patients with sickle cell anaemia in steady state attending the Haematology clinic of a federal tertiary health institution in Ile-Ife, Nigeria. MATERIAL/METHODS Basic demographic data as well as ...

Editorial left ventricular dysfunction in sickle cell disease: the value of an electrocardiographic marker of increased risk of arrhythmia Sickle cell disease is one of the most prevalent genetic diseases worldwide; affecting 1/400 individuals of African descent as well as people of Arab, Indian and Hispanic descents. Abnormalities of cardiovascular function have increasingly been documented in...

Determinations of the acid-base status of 10 adult Jamaican patients with sickle-cell anaemia during "painful crisis" and after recovery showed no evidence of metabolic acidosis in the former, in contrast to reports from elsewhere. These results could explain the failure of alkalis to abort or alter the acute painful episodes of most patients with sickle-cell anaemia.

As sickle cell anaemia (African anaemia; nieniscocytic anaemia) is a rare condition in Asia, we hope that a report on the following three active cases, as well as a number of latent cases showing the sickle cell trait, will raise considerable interest in those parts where the incidence of anaemias among the population is high and where hitherto the response to treatment has proved anything but ...

Acute splenic sequestration crisis in a 20 year old female with homozygous sickle cell anaemia (Hb SS) is described. The resemblance of this complication to that of splenic vein ligation is discussed. This is the first case report known to the author of acute splenic sequestration crisis in an adult with homozygous sickle cell anaemia treated successfully.