Renal abnormalities in sickle cell disease.
TLDR
Sickle cell anemia and related hemoglobinopathies are associated with a large spectrum of renal abnormalities, such as impaired urinary concentrating ability, defects in urinary acidification and potassium excretion, and supranormal proximal tubular function as discussed by the authors.Abstract:
Sickle cell anemia and the related hemoglobinopathies are associated with a large spectrum of renal abnormalities. The patients have impaired urinary concentrating ability, defects in urinary acidification and potassium excretion, and supranormal proximal tubular function. The latter is manifest by increased secretion of creatinine and by reabsorption of phosphorus and beta(2)-microglobulin. Young patients with sickle cell disease (SCD) have supranormal renal hemodynamics with elevations in both effective renal plasma flow (ERPF) and glomerular filtration rate (GFR). These parameters decrease with age as well as following the administration of prostaglandin inhibitors. Proteinuria, a common finding in adults with sickle cell disease, may progress to the nephrotic syndrome. Proteinuria, hypertension, and increasing anemia predict end-stage renal disease (ESRD). While ESRD can be managed by dialysis and/or renal transplantation, there may be an increased rate of complications in renal transplant recipients with SCD. Hematuria is seen in individuals with all of the SCDs as well as with sickle cell trait. In most cases the etiology of the hematuria turns out to be benign. However, there does appear to be an increased association between SCD and renal medullary carcinoma. Therefore, those SCD patients who present with hematuria should initially undergo a thorough evaluation in order to exclude this aggressive neoplasm. Papillary necrosis may occur due to medullary ischemia and infarction. Erythropoietin levels are usually lower than expected for their degree of anemia and decrease further as renal function deteriorates. An abnormal balance of renal prostaglandins may be responsible for some of the changes in sickle cell nephropathy. Acute renal failure is a component of the acute multiorgan failure syndrome (MOFS). Finally, progression of sickle cell nephropathy to ESRD may be slowed by adequate control of hypertension and proteinuria. However, the prevention of the renal complications of SCD will require a cure for this genetic disorder.read more
Citations
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Journal ArticleDOI
Management of Sickle Cell Disease: Summary of the 2014 Evidence-Based Report by Expert Panel Members
Barbara P. Yawn,George R. Buchanan,Araba Afenyi-Annan,Samir K. Ballas,Kathryn L. Hassell,Andra H. James,Lanetta B. Jordan,Sophie Lanzkron,Richard Lottenberg,William J. Savage,Paula Tanabe,Russell E. Ware,M. Hassan Murad,Jonathan C. Goldsmith,Jonathan C. Goldsmith,Eduardo Ortiz,Robinson Fulwood,Ann Horton,Joylene John-Sowah +18 more
TL;DR: Hydxyurea and transfusion therapy are strongly recommended for many individuals with SCD and many other recommendations are based on quality of evidence that is less than high due to the paucity of clinical trials regarding screening, management, and monitoring for individuals withSCD.
Journal ArticleDOI
KDOQI Clinical Practice Guidelines and Clinical Practice Recommendations for Anemia in Chronic Kidney Disease.
TL;DR: This chapter discusses clinical practice guidelines and recommendations for treatment of anemia in children and adults with Kidney Kidney disease, as well as specific cases of patients with HD-CKD.
Journal ArticleDOI
Predicting initiation and progression of chronic kidney disease: Developing renal risk scores
Maarten W. Taal,Barry M. Brenner +1 more
TL;DR: Current data regarding CKD risk factors are reviewed and it is proposed that these risk factors should be used as a basis for developing a renal risk score, analogous to the Framingham risk score for ischemic heart disease, which will allow accurate determination of renal risk in the general population and among CKD patients.
Journal ArticleDOI
Sickle cell disease: renal manifestations and mechanisms
Karl A. Nath,Robert P. Hebbel +1 more
TL;DR: Renal complications and involvement in sickle cell nephropathy (SCN) include altered haemodynamics, hypertrophy, assorted glomerulopathies, chronic kidney disease, acute kidney injury, impaired urinary concentrating ability, distal nephron dysfunction, haematuria, and increased risks of urinary tract infections and renal medullary carcinoma.
Journal ArticleDOI
Pulmonary hypertension associated with sickle cell disease: clinical and laboratory endpoints and disease outcomes.
Laura M. De Castro,Jude Jonassaint,Felicia L. Graham,Allison E. Ashley-Koch,Marilyn J. Telen +4 more
TL;DR: It is confirmed that even relatively mild elevations in pulmonary pressure are associated with high prospective mortality, and pHTN has a high prevalence in all Hb S related syndromes and is associated with increased mortality in SS/Sβ0.
References
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TL;DR: It is suggested that control of glomerular hypertension effectively limits glomersular injury in rats with renal ablation, and the view thatglomerular hemodynamic changes mediate progressive renal injury when nephron number is reduced is supported.
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Book
Sickle Cell Disease
TL;DR: Serjeant as discussed by the authors provides an up-to-date review of the clinical management of sickle cell disease, focusing on three areas of clinical importance: diagnosis, clinical features, and therapy.