scholarly journals Kidney Transplant in a 26-Year-Old Nigerian Patient with Sickle Cell Nephropathy

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
U. H. Okafor ◽  
C. Wachukwu ◽  
P. Emem-Chioma ◽  
F. S. Wokoma
Keyword(s):  
Sickle Cell Disease ◽  
Kidney Transplant ◽  
Sickle Cell ◽  
Disease Patient ◽  
Cell Disease ◽  
Transplant Kidney ◽  
Sickle Cell Nephropathy ◽  
Multiple Challenges ◽  
Stage Renal Disease ◽  
End Stage

Sickle cell nephropathy (SCN) is a common complication of sickle cell disease (SCD). It has variable presentation, ranging from hyposthenuria to end-stage renal disease (ESRD). Management of ESRD in SCD patients is froth with multiple challenges which has potential to impact negatively the outcome of the patient. Kidney transplant is the preferred renal replacement therapy in these patients. The objective of this case study is to report kidney transplant in a Nigerian young man with sickle cell nephropathy and to highlight the outcome and the challenges to kidney transplant in this patient. The index case is a 26-years-old sickle cell disease patient with ESRD complicated with cardiovascular, pulmonary, immunological, and infective challenges. These conditions were controlled, and the patient had a successful live-related kidney transplant. Kidney transplant is a viable option for sickle cell disease patients with ESRD.

scholarly journals Outcome and Challenges of Kidney Transplant in Patients with Sickle Cell Disease

2013 ◽  
Vol 2013 ◽  
pp. 1-8 ◽  
Author(s):  
U. H. Okafor ◽  
E. Aneke
Keyword(s):  
Sickle Cell Disease ◽  
Kidney Disease ◽  
Kidney Transplant ◽  
Sickle Cell ◽  
Cell Disease ◽  
End Stage Kidney Disease ◽  
Transplant Kidney ◽  
Short And Long Term ◽  
Sickle Cell Nephropathy ◽  
End Stage

Sickle cell nephropathy is a common presentation in patients with sickle cell disease. End-stage kidney disease is the most severe presentation of sickle cell nephropathy in terms of morbidity and mortality. Sickle cell disease patients with end-stage kidney disease are amenable to renal replacement therapy including kidney transplant. Kidney transplant in these patients has been associated with variable outcome with recent studies reporting short- and long-term outcomes comparable to that of patients with HbAA. Sickle cell disease patients are predisposed to various haematological, cardiorespiratory, and immunological challenges. These challenges have the potential to limit, delay, or prevent kidney transplant in patients with sickle cell disease. There are few reports on the outcome and challenges of kidney transplant in this group of patients. The aim of this review is to highlight the outcome and challenges of kidney transplant in patients with sickle cell disease.

scholarly journals Early Renal Disease in Children with Sickle Cell Disease from Malawi

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1316-1316
Author(s):  
Jonathan Brett Heimlich ◽  
Godwin Chipoka ◽  
Graham Ellis ◽  
Laila Elsherif ◽  
Emeraghi David ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Renal Disease ◽  
Sickle Cell ◽  
End Stage Renal Disease ◽  
Renal Involvement ◽  
Significant Proportion ◽  
Cell Disease ◽  
Clinical Biomarkers ◽  
Stage Renal Disease ◽  
End Stage

Abstract Sickle cell nephropathy (SCN) is a prevalent complication among adults with sickle cell disease (SCD) and has been observed in younger populations, suggesting potential early renal involvement in pediatric patients. Initial hyperfiltration and albuminuria followed by frank proteinuria, leading to declining GFR and eventual end stage renal disease is assumed to be the typical progression of SCN; however, few clinical biomarkers exist to identify early-stage renal disease. We describe the renal profile in 119 children with SCD at Kamuzu Central Hospital in Lilongwe, Malawi and propose a novel urinary biomarker for the identification of children with early renal disease. Among children with confirmed HbSS disease (females 47.9%; median age 9.0 years, IQR: 5, 11), 21.6% were found to have a urinary albumin to creatinine ratio (UACR) 30mg/g or above. Baseline laboratory and clinical parameters stratified by UACR are shown in Tables 1 and 2. Patients with increased levels of UACR were found to be significantly older, and have significantly elevated plasma levels of lactate dehydrogenase (LDH), total bilirubin, and indirect bilirubin when compared to those without albuminuria (p<0.05). No association was seen between albuminuria and either hemoglobin or plasma hemoglobin. Albuminuria was also significantly associated with elevated levels of nephrin, a urinary marker of glomerular injury (p<0.01). Multivariable logistic regression was used to investigate risk factors that are associated with albuminuria (UACR >= 30). Urine nephrin was significantly associated with albuminuria (regression coefficient estimate: 0.00188, SE: 0.000571, p = 0.0010). Additional analysis using a nephrin cut-point of 293 pg/mL, the median value in the cohort, revealed a 17.8 times greater odds of having albuminuria in children with nephrinuria above this value. These data taken together suggests that a significant proportion of children with SCD in Malawi exhibit renal involvement early in life and could be at risk for worsening nephropathy and end-stage renal disease as they grow older. Our data further indicates that urinary nephrin could be utilized as an early marker of glomerular disease in SCN and possibly prompt earlier intervention in these children. The discordant association of albuminuria with clinical markers of hemolysis suggests that hemolysis may not play a substantial role in the pathogenesis of albuminuria in this population. Increased surveillance of children with SCD for renal complications can ultimately inform management strategies to improve outcomes and increase life expectancy among children with SCD. Disclosures No relevant conflicts of interest to declare.

scholarly journals The morbidity and mortality of end stage renal disease in sickle cell disease

2019 ◽  
Vol 94 (5) ◽  
Author(s):  
Maya Viner ◽  
Jifang Zhou ◽  
David Allison ◽  
Jin Han ◽  
Robert E. Molokie ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Renal Disease ◽  
Sickle Cell ◽  
End Stage Renal Disease ◽  
Morbidity And Mortality ◽  
Cell Disease ◽  
Stage Renal Disease ◽  
End Stage

How I treat renal complications in sickle cell disease

Blood ◽  
2014 ◽  
Vol 123 (24) ◽  
pp. 3720-3726 ◽  
Author(s):  
Claire C. Sharpe ◽  
Swee Lay Thein
Keyword(s):  
Sickle Cell Disease ◽  
Renal Disease ◽  
Sickle Cell ◽  
Symptom Control ◽  
Renal Involvement ◽  
Cell Disease ◽  
Invasive Treatment ◽  
Renal Complications ◽  
Stage Renal Disease ◽  
End Stage

Abstract Renal disease is one of the most frequent and severe complications experienced by patients with sickle cell disease; its prevalence is likely to increase as the patient population ages. We recommend regular monitoring for early signs of renal involvement and a low threshold for the use of hydroxyurea as preventative measures for end-stage renal disease. Once renal complications are detected, a careful assessment of the patient is required to rule out other causes of renal disease. Proteinuria and hypertension should be managed aggressively and the patient referred to a specialist nephrology center when progressive decline in renal function is noted. For the few patients who develop advanced chronic kidney disease, timely planning for dialysis and transplantation can significantly improve outcome, and we recommend an exchange blood transfusion policy for all patients on the transplant waiting list and for those with a functioning graft. Alongside the invasive treatment regimes, it is important to remember that renal failure in conjunction with sickle cell disease does carry a significant burden of morbidity and that focusing on symptom control has to be central to good patient care.

scholarly journals High one year mortality in adults with sickle cell disease and end-stage renal disease

2012 ◽  
Vol 159 (3) ◽  
pp. 360-367 ◽  
Author(s):  
Ann C. McClellan ◽  
Jean-Christophe Luthi ◽  
Janet R. Lynch ◽  
J. Michael Soucie ◽  
Roshni Kulkarni ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Renal Disease ◽  
Sickle Cell ◽  
End Stage Renal Disease ◽  
Cell Disease ◽  
One Year ◽  
Stage Renal Disease ◽  
End Stage

Sheehan's syndrome in a sickle cell disease patient

2019 ◽  
Author(s):  
Ayanbola Adepoju ◽  
Temitope Adeolu ◽  
Ayotunde Ale ◽  
Olatunde Odusan ◽  
Laura Imarhiagbe ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Disease Patient ◽  
Sickle Cell Disease Patient ◽  
Cell Disease ◽  
Sheehan’S Syndrome ◽  
Sheehan's Syndrome
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