Sickle-cell disease and the kidney

2010 ◽  
pp. 4069-4071
Author(s):  
G R Serjeant
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Nocturnal Enuresis ◽  
Renal Medulla ◽  
Vascular Damage ◽  
Tubular Dysfunction ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

The vasa rectae system of the renal medulla is uniquely conducive to sickling, which leads to vascular damage and tubular dysfunction, including an inability to concentrate the urine. Renal presentations include nocturnal enuresis, haematuria (thought to be due to ischaemic lesions of the papillae, including papillary necrosis), and gradually progressive chronic renal failure, which is an important cause of morbidity and death in patients with homozygous sickle-cell disease over the age of 40 years....

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 Sickle cell disease, nephrotic syndrome, and renal failure

2006 ◽  
Vol 69 (7) ◽  
pp. 1276-1280 ◽  
Author(s):  
S.H. Nasr ◽  
G.S. Markowitz ◽  
R.L. Sentman ◽  
V.D. D'agati
Keyword(s):  
Renal Failure ◽  
Nephrotic Syndrome ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease

Erratum

1996 ◽  
Vol 17 (12) ◽  
pp. 425-425
Keyword(s):  
Renal Failure ◽  
Nephrotic Syndrome ◽  
Sickle Cell Disease ◽  
Hiv Infection ◽  
Sickle Cell ◽  
Pneumococcal Vaccine ◽  
Csf Leak ◽  
Cell Disease

The callout on page 339 of the October issue of Pediatrics in Review should read: "Pneumococcal vaccine is recommended for children older than 2 years of age who have sickle cell disease, asplenia, nephrotic syndrome or renal failure, immunosuppression, CSF leak, or HIV infection."

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
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