Kabir O Olaniran1, Nwamaka D Eneanya2, Sagar U Nigwekar1, Xavier F Vela-Parada3, Maureen M Achebe4, Amita Sharma5, Ravi I Thadhani6,7. 1. Division of Nephrology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA. 2. Renal-Electrolyte and Hypertension Division, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA. 3. Department of Medicine, Mt. Sinai Hospital, Icahn School of Medicine, New York, New York, USA. 4. Division of Hematology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA. 5. Division of Pediatric Nephrology, Massachusetts General Hospital for Children, Harvard Medical School, Boston, Massachusetts, USA. 6. Division of Nephrology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA, RTHADHANI@mgh.harvard.edu. 7. Department of Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, California, USA, RTHADHANI@mgh.harvard.edu.
Abstract
BACKGROUND: Compared to the past, patients with sickle cell disease (SCD) currently live longer due to improvements in diagnosis and comprehensive care. Due to these advances, long-term chronic complications pose a greater challenge in the management of patients with SCD. In particular, sickle cell nephropathy (SCN) is associated with significant morbidity and mortality across all age groups. Furthermore, SCN is an understudied condition with relatively few symptoms and therefore requires close surveillance. In this review, we sought to explore the epidemiology, natural history, and treatment options for SCN with an emphasis on the pediatric population. SUMMARY: SCN invariably begins in childhood with evidence of structural changes detected as early as infancy. These indolent changes can progress undetected to advanced chronic kidney disease by late adolescence or early adulthood. The risk factors for progression are not well defined, but significant albuminuria (which is also the most common presentation in childhood) is a key factor in progression. One of the main challenges in understanding SCN in children is the poor correlation between estimated and measured glomerular filtration rates. Another challenge is the lack of large-scale longitudinal studies that track the clinical outcomes of pediatric patients over time. Several studies aim to identify early biomarkers of SCN in children, as albuminuria presents only following significant chronic damage. The utility of angiotensin converting enzyme inhibitors and hydroxyurea in treating albuminuria is addressed here as well as novel treatments that may be of benefit.
BACKGROUND: Compared to the past, patients with sickle cell disease (SCD) currently live longer due to improvements in diagnosis and comprehensive care. Due to these advances, long-term chronic complications pose a greater challenge in the management of patients with SCD. In particular, sickle cell nephropathy (SCN) is associated with significant morbidity and mortality across all age groups. Furthermore, SCN is an understudied condition with relatively few symptoms and therefore requires close surveillance. In this review, we sought to explore the epidemiology, natural history, and treatment options for SCN with an emphasis on the pediatric population. SUMMARY: SCN invariably begins in childhood with evidence of structural changes detected as early as infancy. These indolent changes can progress undetected to advanced chronic kidney disease by late adolescence or early adulthood. The risk factors for progression are not well defined, but significant albuminuria (which is also the most common presentation in childhood) is a key factor in progression. One of the main challenges in understanding SCN in children is the poor correlation between estimated and measured glomerular filtration rates. Another challenge is the lack of large-scale longitudinal studies that track the clinical outcomes of pediatric patients over time. Several studies aim to identify early biomarkers of SCN in children, as albuminuria presents only following significant chronic damage. The utility of angiotensin converting enzyme inhibitors and hydroxyurea in treating albuminuria is addressed here as well as novel treatments that may be of benefit.
Authors: Kabir O Olaniran; Andrew S Allegretti; Sophia H Zhao; Maureen M Achebe; Nwamaka D Eneanya; Ravi I Thadhani; Sagar U Nigwekar; Sahir Kalim Journal: J Am Soc Nephrol Date: 2019-12-06 Impact factor: 10.121
Authors: Oyindamola C Adebayo; Lambertus P Van den Heuvel; Wasiu A Olowu; Elena N Levtchenko; Veerle Labarque Journal: Pediatr Nephrol Date: 2021-05-29 Impact factor: 3.651
Authors: Pamela L Brito; Alisson F Dos Santos; Hanan Chweih; Maria E Favero; Erica M F Gotardo; Juliete A F Silva; Flavia C Leonardo; Carla F Franco-Penteado; Mariana G de Oliveira; Wilson A Ferreira; Bruna C Zaidan; Athanase Billis; Giorgio Baldanzi; Denise A Mashima; Edson Antunes; Sara T Olalla Saad; Fernando F Costa; Nicola Conran Journal: PLoS One Date: 2022-02-03 Impact factor: 3.240