OBJECTIVE To investigate whether attacks of acute chest syndrome affected pulmonary artery pressure in patients homozygous for sickle cell disease. MAIN OUTCOME MEASURES Pulmonary artery pressure, assessed by non-invasive echocardiographic techniques. PATIENTS 20 patients with homozygous sickle cell disease with a history of at least six episodes of acute chest syndrome and in 20 age, sex, ...

Introduction: Sickle cell disease is the most common genetic in world, particularly sub-Saharan Africa. It a protean condition with multiple complications including disturbed iron metabolism. Objectives: To determine prevalence of metabolism abnormalities children homozygous sickle disease, to describe epidemiological, clinical and paraclinical characteristics these identify associated factors....

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...

The characteristics of 214 episodes of invasive bacterial infection among 176 patients with homozygous sickle cell (SS) disease were examined. Streptococcus pneumoniae occurred in 81 episodes, Salmonella spp in 70, Haemophilus influenzae type b in 30, Escherichia coli in 24, and Klebsiella spp in nine. The cumulative incidence showed that S pneumoniae and H influenzae occurred predominantly bef...

Sickel cell disease is common in urban areas of Britain and it is estimated that in London alone there are nearly 2000 patients. One hundred and eighty four patients with sickle cell disease are known to the Central Middlesex Hospital, and 155 of those attend the sickle cell clinic regularly. The commonest cause for admission to hospital is acute painful or vaso-occlusive crisis, which accounts...

Serum samples from 108 unselected Jamaican patients with homozygous sickle cell disease and 116 control subjects with normal haemoglobin were screened for the presence of antiphospholipid antibodies. Slightly increased levels of IgG antiphospholipid antibodies were found in nine patients with sickle cell disease and in none of the control subjects. Serial control samples confirmed the increased...

Amparo Vayáa,∗, Susana Colladoa, Rafael Alisb,c and Maria-Angeles Dası́d aHemorheology and Haemostasis Unit, Service of Clinical Pathology, La Fe University Hospital, Valencia, Spain bResearch Universitary Institute “Dr. Viña Giner”, Molecular and Mitochondrial Medicine, Catholic University of Valencia, “San Vicente Mártir”, Valencia, Spain cFaculty of Medicine, Catholic University of Valencia “...

1. Breen CP, Macdougall IC. Improvement of erythropoietin-resistant anaemia after renal transplantation in patients with homozygous sickle-cell disease. Nephrol Dial Transplant 1998; 13: 2949–2952 2. Tomson CRV, Edmunds ME, Chambers K et al. Effect of recombinant human erythropoietin on erythropoiesis in homozygous sickle-cell anaemia and renal failure. Nephrol Dial Transplant 1992; 7: 817–821 ...

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.