The prevalence of Sickle Nephropathy among children with Sickle Cell Disease
Main Article Content
Keywords
Sickle cell disease, Children, Microalbuminuria, Urine ACR measurements
Abstract
Background: Children with SCD might be at higher risk of progressive renal damage. Hyperfiltration and microalbuminuria are the main criteria of early sickle nephropathy. Urine albumin to creatinine ratio (ACR) is an effective screening tool for detection of sickle nephropathy.
Objective: to assess the prevalence of sickle nephropathy among children with sickle cell anemia using Urine albumin to creatinine ratio (ACR).
Methods: This study included 23 children with HbSS (SCA) and 22 patients with HbSβ0 thalassemia of age 1 to 18 years; forty-five apparently healthy children of comparable age and sex were taken as a control group. All patients were in steady state and had normal kidney functions. All subjects were subjected to full clinical assessment and urine ACR measurements.
Results: The study group showed male sex predominance (m/f ratio 3:2). They were aged 1 to 18 years old with mean age 11.0±3.5 years. Their Mean age at diagnosis was 1.33 ±1.02 years in homozygous SS patients and 1.46 years ± 1.07 years in SB thalassemia patients. Thirty-eight (86%) patients had history of consanguineous marriage and 25 (55.5%) had similar condition in the family. The frequency of CNS vasculopathy among the study group was 20%. Microalbuminuria was present in 33.33% of all study subjects. Seven out of 23 homozygous SS children had microalbuminuria (30.4%) vs. 8 out of 22 with SB-thalassemia (36.4%). Mean ACR was twice higher in the study group compared with the control group (p = 0.002). Mean urine specific gravity was 1019.08±4.17mg/g creat in SS group vs. 1017.14±5.16 in SB thalassemia group (p=0.170). Mean A/C ratio was 25.56±20.95 mg/g among sickle SS group of patients vs. 31.09±30.91 among sickle beta thalassemia group of patients (p=0.617)
Conclusions: ACR is higher in children with SCD than in controls. Microalbuminuria is a common finding in children with SCD. Children with SB-thalassemia might be at higher risk of sickle nephropathy.
References
2. McKie KT, Hanevold CD, Hernandez C, Waller JL, Ortiz L, McKie KM. Prevalence, prevention, and treatment of microalbuminuria and proteinuria in children with sickle cell disease. Journal of pediatric hematology/oncology. 2007 Mar 1;29(3):140-4.
3. Hariri E, Mansour A, El Alam A, Daaboul Y, Korjian S, Aoun Bahous S. Sickle cell nephropathy: an update on pathophysiology, diagnosis, and treatment. International Urology and Nephrology. 2018 Jun;50(6):1075-83.
4. Niss O, Lane A, Asnani MR, Yee ME, Raj A, Creary S, Fitzhugh C, Bodas P, Saraf SL, Sarnaik S, Devarajan P. Progression of albuminuria in patients with sickle cell anemia: a multicenter, longitudinal study. Blood advances. 2020 Apr 14;4(7):1501-11.
5. Schwartz GJ, Schneider MF, Maier PS, Moxey-Mims M, Dharnidharka VR, Warady BA, Furth SL, Muñoz A. Improved equations estimating GFR in children with chronic kidney disease using an immunonephelometric determination of cystatin C. Kidney international. 2012 Aug 2;82(4):445-53.
6. Thompson J, Reid M, Hambleton I, Serjeant GR. Albuminuria and renal function in homozygous sickle cell disease: observations from a cohort study. Archives of Internal Medicine. 2007 Apr 9;167(7):701-8.
7. Ghobrial EE, Abdel-Aziz HA, Kaddah AM, Mubarak NA. Urinary transforming growth factor β-1 as a marker of renal dysfunction in sickle cell disease. Pediatrics & Neonatology. 2016 Jun 1;57(3):174-80.
8. Alvarez O, Montane B, Lopez G, Wilkinson J, Miller T. Early blood transfusions protect againstmicroalbuminuria in children with sickle cell disease. Pediatric blood & cancer. 2006 Jul;47(1):71-6.
9. Asnani MR, Lynch ON, Reid ME. Determining glomerular filtration rate in homozygous sickle cell disease: utility of serum creatinine based estimating equations. PloS one. 2013 Jul 19;8(7):e69922.
10. Aygun B, Mortier NA, Smeltzer MP, Hankins JS, Ware RE. Glomerular hyperfiltration and albuminuria in children with sickle cell anemia. Pediatric nephrology. 2011 Aug;26(8):1285-90.
11. Iwalokun BA, Iwalokun SO, Hodonu SO, Aina OA, Agomo PU. Evaluation of microalbuminuria in relation to asymptomatic bacteruria in Nigerian patients with sickle cell anemia. Saudi Journal of Kidney Diseases and Transplantation. 2012 Nov 1;23(6):1320.