Some haematological and rheological features were compared in 27 age and sex matched pairs of patients (15 male, 12 female) with homozygous sickle cell (SS) disease with and without proliferative sickle retinopathy (PSR). Significant haematological differences between the groups were a higher haemoglobin and a lower fetal haemoglobin in PSR positive males and a higher MCHC in PSR positive femal...

The determinants of nocturnal enuresis in homozygous sickle cell (SS) disease have been investigated in 16 enuretic and 16 age and sex matched non-enuretic children. Overnight fluid deprivation tests (8pm-8am) demonstrated no significant difference in maximum urine osmolality or urine volumes, although the latter tended to be higher in the enuretic children. Maximum functional bladder capacity,...

Analysis of the growth abnormalities in sickle cell disease has been limited by the lack of longitudinal observations in individuals, and by an inability to quantitate the observed patterns. To investigate the timing and pattern of the adolescent growth spurt, longitudinal observations of height from the Jamaican cohort study were fitted to a mathematical model of growth (Preece-Baines model 1)...

Serum and urinary urate concentrations were studied in 44 patients with homozygous sickle cell (SS) disease, and in 27 controls with normal haemoglobin. Hyperuricaemia (>0.39 mmol/l (6.5 mg/100 ml)) occurred in 41% of SS patients and inversely correlated with renal urate clearance but not with indices of bone marrow turnover. Higher serum urate concentrations occurred in patients with proteinur...

BACKGROUND AND PURPOSE Sickle cell disease (SCD) is associated with cerebral hyperperfusion and an increased risk of stroke. Also, both recurrent microvascular obstruction and chronic hemolysis affect endothelial function, potentially interfering with systemic and cerebral blood flow control. We addressed the question whether cerebrovascular control in patients with SCD is affected and related ...

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 ...

Sickle cell anemia (SCD) is one of the important health problems in Basrah, southern Iraq. which probably most common known hereditary blood disorder. patients with sickle disorders often suffer from chronic hemolytic anemia, causes bone marrow hyperplasia too. The present study aimed to evaluate severity impairment different types patients. Patients SCA (n=120) 60 males and females were on fol...

A 22-year-old female with factitious sickle cell anemia and recurrent painful crises is described. Because she had sickle cell trait and iron deficiency anemia, she could successfully feign the symptoms of homozygous sickle cell anemia. The identification of this syndrome in patients with genetic disorders is presented.

Extracorporeal membrane oxygenation is used in Sickle cell disease patients is unclear. We present a case of a 6 years old girl with homozygous hemoglobin SS disease complicated with chest syndrome. Oxygenation was maintained with two days of VA extracorporeal membrane oxygenation and 30 days of VV ECMO.