Risk Factors and Complications Among Pediatric Patients With Sickle Cell Anemia: A Single Tertiary Center Retrospective Study

Abstract

Background and objective Sickle cell anemia (SCA) is one of the common genetic diseases in the Kingdom of Saudi Arabia (KSA). This disease results from a genetic mutation that causes malformation of the red blood cells (RBCs), leading to various systemic complications, including vaso-occlusive crisis (VOC), acute chest syndrome (ACS), osteomyelitis, avascular necrosis (AVN), and stroke, to name a few. The leading cause of mortality in SCA is these systemic complications rather than the disease itself. Understanding the risk factors of these complications can help reduce mortality in these patients and improve their quality of life. In this study, we aimed to determine the risk factors of SCA complications among pediatric patients with SCA at King Abdulaziz University Hospital (KAUH) in Jeddah, KSA. Methods This retrospective study was carried out from January 2012 till June end 2019. It was conducted among pediatric patients with SCA. Patients were screened for eligibility, and we excluded those with thalassemia and those who had a medical history of chronic diseases. Data were collected from patients’ electronic medical records. Results The study included 102 pediatric patients with SCA; their mean age was 7.88 ±4.22 years; almost half of them were females (56%) and 44% were males. The dominant body mass index (BMI) classification among them was normal (49%). Urinary tract infection (UTI) was the most common complication with 38 cases followed by VOC with 32 cases. Other complications observed were ACS (25.5%) followed by stroke (15.7%). HbSS was the most prominent genotype among these patients, and it was associated with a higher rate of complications. However, there was no significant relationship between genotype and patients developing complications. Finally, patients with high white blood cell (WBC) counts, elevated systolic blood pressure (SBP), and hypoxia developed more complications, and there was a significant relationship between these conditions and the development of complications (p<0.05). Conclusion Based on our findings, patients with high WBC count, elevated SBP, and hypoxia are at greater risk of developing complications. Accordingly, healthcare providers should consider putting in place all measures required to provide a good quality of life for these patients, including raising awareness about the risk factors that lead to these complications, appropriate immunizations, and precautionary measures to promote these patients' welfare.

Pediatric Sickle Cell Disease and Stroke: A Literature Review

“…In Kuwait, the prevalence of stroke is 1.4% in children [30], and in Shiraz, Iran, it seems to be low among children with SCD. When adults are considered, the stroke prevalence is high in Saudi Arabia's provinces (~7%) [31][32][33][34].…”

Epidemiology of Stroke in Sickle Cell Disease

“…One paediatric study from high and low altitude regions of SouthWest Saudi Arabia reported 8/400 (2%) over a 3 year period [ 51 ] but 9/90 (10%) of a series of children from Medina had a clinical stroke, with eight having an abnormal CT scan [ 52 ]. When adults are included, the prevalences of stroke in the Western, SouthWestern (7.5%) and Eastern (6%) provinces of Saudi Arabia are high [ 53 , 54 , 55 , 56 ].…”

Hematopoietic Stem Cell Transplantation Stabilizes Cerebral Vasculopathy in High-Risk Pediatric Sickle Cell Disease Patients: Evidence From a Referral Transplant Center

“…From a hospital-based study of SCD in the western part of Saudi Arabia, the incidence of overt stroke in children was reported to be 9.4% in a predominantly African (severe) haplotype [ 7 - 9 ]. While in Eastern Province this statistic is around 6% [ 10 ]. It is expected to be lower considering its less severe phenotype due to the high hemoglobin F percentage (Arab-Indian haplotype) compared to the severe African type seen in the Southwestern region, which all of our patient cohort represents [ 7 ].…”