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Glomerular lesions in patients with sickle cell disease
Gwenola Maigne1, Sophie Ferlicot, Frederic Galacteros
1From Internal Medicine Unit (GW, SS, OL), Pathology Department (SF), and Nephrology and Renal Transplantation Department (AD), APHP, Hôpital Kremlin Bicêtre, Université Paris 11, Le Kremlin Bicêtre; Sickle Cell Disease Center (FG) and Internal Medicine Unit (BG), APHP, Hôpital Henri Mondor, Université Paris 12, Créteil; Nephrology and Renal Transplantation Department (PL, VA), APHP, Hôpital Henri Mondor, and Institut Francilien de recherche en Néphrologie et Transplantation (IFRNT), INSERM U 955, Université Paris 12, Créteil; Nephrology Department (XB), Hôpital de Montreuil, Montreuil; Nephrology Department (TU), APHP, Hôpital Trousseau, UPMC Université Paris 06, Paris; Pediatric Nephrology Department (PN), APHP, Hôpital Necker-Enfants Malades, Université Paris Descartes, Paris; Nephrology Department (PR), APHP, Hôpital Tenon, UPMC Université Paris 06, Paris; France.
Sickle cell disease (SCD) causes various kidney lesions, with poor prognosis regardless of type. Early treatment is crucial for kidney health in SCD patients.
Area of Science:
- Nephrology
- Hematology
- Pathology
Background:
- Sickle cell disease (SCD) is a growing cause of chronic kidney disease.
- The range of glomerular lesions and their mechanisms in SCD are not well understood.
Purpose of the Study:
- To describe the spectrum of glomerular lesions in SCD patients.
- To investigate the role of hypoxic markers in SCD-related kidney disease.
Main Methods:
- Review of 18 renal biopsies from SCD patients with glomerular involvement.
- Histopathologic analysis and immunohistochemical study of hypoxic markers (iNOS, nitrotyrosine, HIF-1 alpha).
Main Results:
- Four histopathologic variants identified: FSGS (39%), MPGN (28%), TM (17%), and SCD glomerulopathy (17%).
- Macroalbuminuria was universal; impaired renal function was observed in only 6 patients.
- 50% of patients developed chronic kidney disease during follow-up; hypoxic markers were not significantly induced.
Conclusions:
- SCD is associated with diverse glomerular lesions, all carrying a poor renal prognosis.
- Early renoprotective strategies are essential for managing SCD-related kidney disease.
- Hypoxia may not be a primary driver of established glomerular injury in SCD, but its early role warrants further study.
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