Renal outcomes in pediatric patients with sickle cell disease: a single center experience in Saudi Arabia

Dania A Monagel; Shatha S Algahtani; Lian A Karawagh; Wafa D Althubaity; Sara A Azab; Deena F Haneef; Naglla Elimam

doi:10.3389/fped.2023.1295883

Renal outcomes in pediatric patients with sickle cell disease: a single center experience in Saudi Arabia

Front Pediatr. 2023 Dec 15:11:1295883. doi: 10.3389/fped.2023.1295883. eCollection 2023.

Authors

Dania A Monagel^{1

2

3}, Shatha S Algahtani^#¹, Lian A Karawagh^#¹, Wafa D Althubaity^#¹, Sara A Azab^#¹, Deena F Haneef⁴, Naglla Elimam³

Affiliations

¹ College of Medicine, King Saud bin Abdul-Aziz University for Health Sciences, Jeddah, Saudi Arabia.
² King Abdullah International Medical Research Center, Jeddah, Saudi Arabia.
³ Department of Oncology, Ministry of the National Guard-Health Affairs, Jeddah, Saudi Arabia.
⁴ Center of Excellence in Genomic Medicine Research (CEGMR), King Abdulaziz University, Jeddah, Saudi Arabia.

^# Contributed equally.

Abstract

Background: Sickle cell nephropathy (SCN) is a significant complication of sickle cell disease (SCD) with an asymptomatic onset in childhood and potential progression to chronic kidney disease (CKD). The clinical findings of SCN include hyposthenuria, hematuria, proteinuria, hyperfiltration, and CKD. Data on renal manifestation among patients with SCD in Saudi Arabia is lacking. Therefore, this study aimed to evaluate renal outcomes in patients with SCD who visited a hematology clinic at the National Guard Hospital, Jeddah.

Methods: We conducted a retrospective chart review of renal complications in patients with SCD who are within 0-14 years of age and on regular follow-ups at the pediatric hematology clinic in King Abdulaziz Medical City-Jeddah, Saudi Arabia.

Results: Among the 140 patients with SCD, 99 met the inclusion criteria. The median age at diagnosis was 18 (1-108) months. Two SCD phenotypes were observed, with 82 (83%) patients having sickle cell anemia (HbSS) and 17 (17%) having HbS/B⁺ thalassemia. Of the total patients, 92 (93%) were administered hydroxyurea (HU), with a median starting age of 48 (9-168) months. The most common renal complication observed during routine urinalysis was hematuria (38%), followed by proteinuria (11%). After stratifying the sample into four age groups (0-3 years old, 4-7 years old, 8-11 years old, and 12-14 years old), the mean glomerular filtration rate (GFR) values were 96.16, 101.36, 112.69, and 120.11 ml/min/1.73 m² respectively. Renal imaging revealed abnormal findings in 27 (29%) patients. The most common abnormality observed on US was increased echogenicity (43%).

Conclusion: SCN is a significant complication of SCD. In this study, we assessed the renal outcomes in pediatric patients with SCD. After analyzing the clinical findings of SCN, we concluded that the presence of renal complications in pediatric patients presented a progressive pattern.

Keywords: complications; nephropathy; pediatric; renal; sickle cell disease.

Grants and funding

The author(s) declare that no financial support was received for the research, authorship, and/or publication of this article.