scholarly journals Haematological factors associated with proliferative retinopathy in homozygous sickle cell disease.

1981 ◽  
Vol 65 (1) ◽  
pp. 29-35 ◽  
Author(s):  
R. J. Hayes ◽  
P. I. Condon ◽  
G. R. Serjeant
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Proliferative Retinopathy ◽  
Cell Disease ◽  
Factors Associated ◽  
Homozygous Sickle Cell Disease

scholarly journals Blood rheology and proliferative retinopathy in homozygous sickle cell disease.

1986 ◽  
Vol 70 (7) ◽  
pp. 522-525 ◽  
Author(s):  
B E Serjeant ◽  
K P Mason ◽  
R W Acheson ◽  
G H Maude ◽  
J Stuart ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Blood Rheology ◽  
Proliferative Retinopathy ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

Increased Procoagulant Activity of Red Blood Cells from Patients with Homozygous Sickle Cell Disease and β-Thalassemia

1996 ◽  
Vol 76 (03) ◽  
pp. 322-327 ◽  
Author(s):  
Dominique Helley ◽  
Amiram Eldor ◽  
Robert Girot ◽  
Rolande Ducrocq ◽  
Marie-Claude Guillin ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Annexin V ◽  
Mean Value ◽  
Procoagulant Activity ◽  
Dependent Manner ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

SummaryIt has recently been proved that, in vitro, red blood cells (RBCs) from patients with homozygous β-thalassemia behave as procoagulant cells. The procoagulant activity of β-thalassemia RBCs might be the result of an increased exposure of procoagulant phospholipids (i. e. phosphatidylserine) in the outer leaflet of the membrane. In order to test this hypothesis, we compared the catalytic properties of RBCs of patients with β-thalassemia and homozygous sickle cell disease (SS-RBCs) with that of controls. The catalytic parameters (Km, kcat) of prothrombin activation by factor Xa were determined both in the absence and in the presence of RBCs. The turn-over number (kcat) of the reaction was not modified by normal, SS- or (3-thalassemia RBCs. The Km was lower in the presence of normal RBCs (mean value: 9.1 µM) than in the absence of cells (26 µM). The Km measured in the presence of either SS-RBCs (mean value: 1.6 µM) or β-thalassemia RBCs (mean value: 1.5 pM) was significantly lower compared to normal RBCs (p <0.001). No significant difference was observed between SS-RBCs and p-thalassemia RBCs. Annexin V, a protein with high affinity and specificity for anionic phospholipids, inhibited the procoagulant activity of both SS-RBCs and (3-thalassemia RBCs, in a dose-dependent manner. More than 95% inhibition was achieved at nanomolar concentrations of annexin V. These results indicate that the procoagulant activity of both β-thalassemia RBCs and SS-RBCs may be fully ascribed to an abnormal exposure of phosphatidylserine at the outer surface of the red cells.

scholarly journals Clinical findings associated with homozygous sickle cell disease in the Barbadian population – do we need a national SCD registry?

2014 ◽  
Vol 7 (1) ◽  
Author(s):  
Kim R Quimby ◽  
Stephen Moe ◽  
Ian Sealy ◽  
Christopher Nicholls ◽  
Ian R Hambleton ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Findings ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

scholarly journals Concurrent homozygous sickle‐cell disease and severe haemophilia A: Thromboelastography profiles

Haemophilia ◽  
2019 ◽  
Vol 25 (2) ◽  
Author(s):  
Ahmar U. Zaidi ◽  
Latha Rao ◽  
Michael U. Callaghan ◽  
Madhvi Rajpurkar ◽  
Wendy Hollon ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

scholarly journals Factors affecting prepubertal growth in homozygous sickle cell disease.

1996 ◽  
Vol 74 (6) ◽  
pp. 502-506 ◽  
Author(s):  
A Singhal ◽  
J Morris ◽  
P Thomas ◽  
G Dover ◽  
D Higgs ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Factors Affecting ◽  
Homozygous Sickle Cell Disease

scholarly journals Improved survival in homozygous sickle cell disease: lessons from a cohort study

BMJ ◽  
1995 ◽  
Vol 311 (7020) ◽  
pp. 1600-1602 ◽  
Author(s):  
A. Lee ◽  
P. Thomas ◽  
L. Cupidore ◽  
B. Serjeant ◽  
G. Serjeant
Keyword(s):  
Cohort Study ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

scholarly journals Factors associated with undernutrition among children with sickle cell disease attending the sickle cell clinic in Mulago National Referral Hospital, Uganda

2019 ◽  
Author(s):  
Rosta Asiimwe ◽  
Rornald Muhumuza Kananura ◽  
Richard Kajjura ◽  
Adoke Yeka
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Older Age ◽  
Referral Hospital ◽  
Childhood Mortality ◽  
Mulago Hospital ◽  
Cell Disease ◽  
Factors Associated ◽  
Mulago National Referral Hospital ◽  
National Referral Hospital

Abstract Background Sickle cell disease (SCD) is among the neglected non-communicable diseases, which significantly contributes to early childhood mortality. In Uganda, over 20,000 children are estimated to be sicklers. Undernutrition is common among children with SCD and contributes to increased morbidity and mortality. There is paucity of data on prevalence of undernutrition and associated factors in Uganda. Objective To assess the extent of undernutrition and related factors among children aged 5-12 years with SCD attending the sickle cell clinic at Mulago hospital, Uganda. Methods A total of 263 children with SCD attending the sickle cell clinic at Mulago National Referral hospital were recruited consecutively between May and June 2017. The nutritional status of the children was assessed by weight-for-age, BMI-for-age, and height-for-age z-scores calculated using STATA in accordance with WHO 2007 growth standards. Binomial regression was conducted to assess the predictors of undernutrition. Results About 20.2%, 11.4%, and 13.7% of the children were underweight, wasted and stunted respectively. Wasting was significantly associated with older age (10-12 years) (AOR=4.20, CI=2.18-8.10) and living in a female headed household (AOR=0.43, CI=0.19-0.99). Stunting was significantly associated with older age (10-12 years) (AOR=2.90, CI=1.39-6.06). Underweight was significantly associated with older age (10-12 years) (AOR=2.23, CI=1.05-5.16). Conclusion Underweight, wasting and stunting were prevalent among children with SCD attending Mulago hospital. The factors associated with undernutrition were older age and living in a female headed household.

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