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Saudi Journal of Kidney Diseases and Transplantation
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LETTER TO EDITOR Table of Contents   
Year : 2004  |  Volume : 15  |  Issue : 2  |  Page : 174-175
End-Stage Sickle Cell Nephropathy: Determinants of Reduced Survival of Patients on Long-term Hemodialysis

Division of Nephrology, Post-graduate Department of Medicine, Hofuf, Al-Hasa-31982, Saudi Arabia

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How to cite this article:
Saxena AK, Panhotra B R, Al-Arabi Al-Ghamdi AM. End-Stage Sickle Cell Nephropathy: Determinants of Reduced Survival of Patients on Long-term Hemodialysis. Saudi J Kidney Dis Transpl 2004;15:174-5

How to cite this URL:
Saxena AK, Panhotra B R, Al-Arabi Al-Ghamdi AM. End-Stage Sickle Cell Nephropathy: Determinants of Reduced Survival of Patients on Long-term Hemodialysis. Saudi J Kidney Dis Transpl [serial online] 2004 [cited 2021 Apr 14];15:174-5. Available from: https://www.sjkdt.org/text.asp?2004/15/2/174/32902
To the Editor:

The development of chronic renal failure (CRF) is a predictor of poor outcome in patients with Sickle Cell Disease (SCD). [1],[2],[3],[4],[5] It occurs in 4-20% of the patients with SCD; the prevalence rates increase with advancing age, reaching up to 33% among those above 40-years of age. [4] The presence of CRF significantly shortens the survival of these patients, with a median life expectancy of 27 years, as opposed to 51 years for those without renal failure. The median survival of patients with SCD becomes only four years from the time a diagnosis of CRF is made. [4] While renal replacement therapy in the form of hemodialysis (HD) and renal transplantation generally remains the standard management of patients with-end stage sickle cell nephro­pathy; very little is known regarding long-term outcome in terms of mortality and its determinants in these patients.

A retrospective cohort study was carried out among 203 patients (11 with SCD, Group I and, 192 with ESRD due to diverse etiologies, Group II) attending the HD facility between January 1992 to January 1999, at The King Fahad Hospital and Tertiary Care Center, Hofuf, Eastern Province of Saudi Arabia.

Factors that frequently affect the morbidity and mortality of patients on long-term HD such as age, gender, duration on dialysis, number of units of blood transfused, presence of hepatitis B and /or hepatitis C infection (HBV and HCV) and number of episodes of vascular access-related septicemia (VRS), were documented and compared between the two groups.

Patients in Group I recorded a higher require­ment of blood transfusion than those in group II (13.7 ± 6 versus 8.2 ± 3.3 units, p<0.05). Group I patients also had a significantly higher prevalence of HBV (18.2 %) than those in Group II (4.64 %, p< 0.002). Similarly, the prevalence of anti-HCV antibodies in Groups I and II was 63.6 % and 44.3% respectively, p< 0.004). Likewise, a higher prevalence of VRS was recorded among patients of Group I in comparison to that of Group II (2.59 vs. 1.19 episodes / 100 patient months respecti­vely, p<0.0001). Significantly higher mortality was noted in Group I (11.59% per year) when compared to Group II (5.87% per year, p< 0.001); also, death occurred at a comparatively younger age (31 ± 8.2 vs. 47.8 ± 18 years, p<0. 0.021) and after an appreciably shorter duration on HD (27 ± 5.6 vs. 44.2 ± 12.3 months, p<0.008).

Evidently, ESRD patients with SCD had a poor outcome on long-term HD, in terms of survival (higher mortality at a significantly younger age and shorter period on dialysis) in comparison to those without SCD. The early onset of ESRD, a higher prevalence of HBV and HCV infections along with the significantly increased prevalence of VRS, seem to be the important potential deter­minants of the reduced survival of SCD patients seen in our study. Low survival rates are largely related to the bacterial infections (including VRS), in this asplenic population. [5] Additionally, oxidative stress caused by HD filters may affect cell mediated immunity adversely, in an already immuno-compro­mised ESRD population with SCD. [6] Higher mortality, in patients with SCD on HD, has also been reported due to hepatic failure related to iron overload and chronic hepatitis (HBV and HCV), as HD creates a high-risk environment for the transmission of HCV in association with the obligatory need for vascular access sites and extracorporeal blood circulation. [4],[5],[7]

Data from national registries, both in children and adults, indicate a five-year survival after renal transplantation of 18% which is at least as good as the reported 17% survival at five years on maintenance HD. [8],[9],[10] In registry data from 1984 to 1996, the one-year allograft survival in SCD patients was identical to age matched Afro-American transplant recipients, although the three-year survival of 48% was significantly less than the 60% seen in the control population. [8] Taking into consideration the reasonably superior survival rates of renal transplantation in comparison to those on long-term HD, the patients with end-stage sickle cell nephropathy on HD may be encouraged to take up renal transplantation in priority over those without SCD.

   References Top

1.Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med 1994;330:1639-44.  Back to cited text no. 1    
2.Thomas AN, Pattison C, Serjeant GR. Causes of death in sickle-cell disease in Jamaica. Br Med J 1982;285:633-5.  Back to cited text no. 2    
3.Saborio P, Scheinman JI. Sickle cell nephropathy. J Am Soc Nephrol 1999;10: 187-92.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Rodgers GP, Walker EC, Podgor MJ. Mortality in Sickle cell disease. N Engl J Med 1994;331:1022-3.  Back to cited text no. 4  [PUBMED]  
5.Powars DR, Elliott-Mills DD, Chan L, et al. Chronic renal failure in sickle cell disease: risk factors, clinical course and mortality. Ann Intern Med 1991;115:614-20.  Back to cited text no. 5  [PUBMED]  
6.Descamps-Latscha B, Jungers P, Witko­Sarsat V. Immune system dysregulation in uremia: role of oxidative stress. Blood Purif 2002;20:481-4.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Saxena AK, Panhotra BR, Sundaram DS. The role, the type of vascular access plays in the transmission of hepatitis C virus in a high prevalence hemodialysis unit. J Vasc Access 2002;3(4):158-63.  Back to cited text no. 7    
8.Warady BA, Sullivan EK. Renal transplantation in children with sickle cell disease: a report of the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS). Pediatr Transplant 1998;2:130-3.  Back to cited text no. 8  [PUBMED]  
9.Ojo AO, Govaerts TC, Schmouder RL et al. Renal transplantation in end-stage sickle cell nephropathy. Transplantation 1999;67: 291-5.  Back to cited text no. 9    
10.Brennan DC, Lippmann BJ, Shenoy S, et al. Living unrelated renal transplantation for sickle cell nephropathy. Transplantation 1995;59:794-5.  Back to cited text no. 10  [PUBMED]  

Correspondence Address:
Anil K Saxena
Division of Nephrology, Post-graduate Department of Medicine, Hofuf, Al-Hasa-31982
Saudi Arabia
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PMID: 17642772

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