Enalapril and hydroxyurea therapy for children with sickle nephropathy

Pediatric Blood and Cancer - Tập 45 Số 7 - Trang 982-985 - 2005
Courtney D. Fitzhugh1, Delbert R. Wigfall2, Russell E. Ware3
1Department of Internal Medicine, Duke University Medical Center, Durham, North Carolina, USA
2Division of Nephrology, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina.
3Division of Hematology, St. Jude Children's Research Hospital, Memphis, Tennessee.

Tóm tắt

AbstractProteinuria in children with sickle cell anemia (SCA) is an early sign of sickle nephropathy, and portends the development of nephrotic syndrome and chronic renal failure. Enalapril has been shown to reduce proteinuria in adult patients with SCA, but the potential benefits of hydroxyurea in this clinical setting have not been reported. A single institution retrospective analysis was performed. Children with sickle nephropathy were identified, and the laboratory effects of enalapril and hydroxyurea therapy were evaluated in children with substantial proteinuria. Three children developed proteinuria at 8 ± 1 years of age. Pre‐treatment laboratory studies included a low serum albumin (2.8 ± 0.8 g/dl) and a highly elevated urine protein/creatinine ratio (6.9 ± 3.7, normal <0.2). Enalapril treatment for 3.0 ± 1.3 years normalized serum albumin (3.9 ± 0.3 g/dl) without significant changes in serum potassium, serum creatinine, or systolic blood pressure. However, urine protein/creatinine remained elevated in the nephrotic range (1.6 ± 0.7). The addition of hydroxyurea therapy for 3.5 ± 1.2 years increased fetal hemoglobin levels (7.0 ± 3.6% to 21.0 ± 3.2%) and was associated with a near‐normal urine protein/creatinine ratio (0.5 ± 0.1). Enalapril therapy for children with sickle nephropathy reduces urinary protein excretion and normalizes serum albumin. Hydroxyurea therapy may further normalize the urine protein/creatinine ratio. Combination therapy should be tested prospectively in children with sickle nephropathy. © 2005 Wiley‐Liss, Inc.

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Tài liệu tham khảo

10.7326/0003-4819-115-8-614

Falk RJ, 1994, Sickle cell nephropathy, Adv Nephrology, 23, 133

10.1046/j.1523-1755.2000.00806.x

10.1046/j.1523-1755.2002.00363.x

10.1067/mpd.2000.105998

10.1159/000167444

10.1056/NEJM199406093302303

10.5414/CNP58009

10.1097/00007890-199901270-00018

10.1056/NEJM199204023261402

10.1016/S0002-9343(99)80341-6

10.1016/S0002-9343(98)00056-4

10.1056/NEJM199505183322001

Kinney TR, 1999, Safety of hydroxyurea in children with sickle cell anemia: Results of the HUG‐KIDS study, a phase I/II trial, Blood, 94, 1550

10.1182/blood-2003-07-2475

10.1016/j.jpeds.2004.04.058

Zimmerman SA, 2004, Increased glomerular filtration rate as an early marker of sickle nephropathy in young children with sickle cell anemia (abstract), Am Soc Pediatr Hematol/Oncol

10.1007/s004670050491

10.2165/00128072-200204010-00004

10.1034/j.1600-0609.2002.02775.x