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 ◽  
...  
Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1316-1316
Author(s):  
Jonathan Brett Heimlich ◽  
Godwin Chipoka ◽  
Graham Ellis ◽  
Laila Elsherif ◽  
Emeraghi David ◽  
...  

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.


2019 ◽  
Vol 94 (5) ◽  
Author(s):  
Maya Viner ◽  
Jifang Zhou ◽  
David Allison ◽  
Jin Han ◽  
Robert E. Molokie ◽  
...  

Blood ◽  
2014 ◽  
Vol 123 (24) ◽  
pp. 3720-3726 ◽  
Author(s):  
Claire C. Sharpe ◽  
Swee Lay Thein

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.


2017 ◽  
Vol 32 (suppl_3) ◽  
pp. iii35-iii35
Author(s):  
Nynke Halbesma ◽  
Eve Miller-Hodges ◽  
Gurbey Ocak ◽  
Sarah Wild ◽  
Friedo Dekker ◽  
...  

1981 ◽  
Vol 2 (1_suppl) ◽  
pp. 6-10 ◽  
Author(s):  
Pablo Amair ◽  
Ramesh Khanna ◽  
Bernard Leibel ◽  
Andreas Pierratos ◽  
Stephen Vas ◽  
...  

Twenty diabetics with end-stage renal disease who had never previously received dialysis treatment were treated with continuous ambulatory peritoneal dialysis for periods of two to 36 months (average, 14.5). Intraperitoneal administration of insulin achieved good control of blood sugar Even though creatinine clearance decreased significantly (P = 0.001), contro of blood urea nitrogen and serum creatinine was adequate. Hemoglobin and serum albumin levels increased significantly (P = 0.005 and 0.04 respectively). Similarly, there was a significant increase in serum triglycerides and alkaline phosphatase (P = 0.02 and 0.05). Blood pressure became normal without medications in all but one of the patients. Retinopathy, neuropathy, and osteodystrophy remained unchanged. Peritonitis developed once in every 20.6 patient-months a rate similar to that observed in nondiabetics. The calculated survival rate was 92 per cent at one year; the calculated rate of continuation on ambulatory peritoneal dialysis was 87 per cent.


1991 ◽  
Vol 21 (4) ◽  
pp. 343-354 ◽  
Author(s):  
Rolf A. Peterson ◽  
Paul L. Kimmel ◽  
Carol R. Sacks ◽  
Mary Louise Mesquita ◽  
Samuel J. Simmens ◽  
...  

A role of depression in affecting outcome in patients with end stage renal disease (ESRD) has been suggested but few have assessed psychological parameters and medical factors thought to influence survival simultaneously and prospectively. To assess whether depression or perception of illness influences survival in patients treated for ESRD, we prospectively evaluated fifty-seven patients with ESRD treated with hemodialysis (HD, n = 43) or continuous ambulatory peritoneal dialysis (CAPD, n = 14). Patients were interviewed and completed the Beck Depression Inventory (BDI) and the Illness Effects Questionnaire (IEQ). An ESRD severity coefficient was used to measure chronic illness severity. A cognitive item subset of the BDI (CDI) was used as an additional measure of depression. One and two years later, records were examined to determine survival. When initial results of the assessment of survivors and non-survivors were compared, at one year follow-up, there were no differences in mean age, duration of dialysis, severity scores, BDI or IEQ scores. The initial mean CDI scores in the group of non-survivors, however, were significantly greater than the scores in the survivor group. At two year follow-up, CDI scores were significantly different between groups, and were significant in a hazards regression. Disease severity, age and duration of dialysis were also significantly related to mortality at two year follow-up. We conclude cognitive depression is an important, early, indicator of grave prognosis in patients treated for ESRD. Early recognition of and therapeutic efforts directed toward the treatment of depression might modify outcome in ESRD patients.


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