Table 1.
Demographics and baseline characteristics of the included patients (n=42)
| Study variables | Total population (n=42), n (%) |
|---|---|
| Demographics | |
| Gender | |
| Male: female | 22:20 (52.4:47.6) |
| Nationality | |
| Saudi | 41 (97.6) |
| Genotype | |
| Hgb SS | 29 (69) |
| Hgb Sβ0 thalassemia | 9 (21.4) |
| Hgb Sα thalassemia | 4 (9.5) |
| Age at the time of first ACS diagnosis, mean±SD | 6.62±3.38 |
| Age grouping at the time of first ACS diagnosis | |
| Infants (≤1 year) | 2 (4.8) |
| Toddlers (1–3 years) | 8 (19) |
| Children (4–14 years) | 32 (76.2) |
| Electrophoresis at SCD diagnosis (mean±SD)* | |
| HbS | 71.69±12.57 |
| HbF | 24.94±12.6 |
| HbA2 | 2.16±1.19 |
| Number of VOC admissions (years), median (IQR) | 2 (1–3) |
| SCD-related hospitalizations (years), median (IQR) | 3 (2–4) |
| SCD-related adverse events encountered throughout their pediatric follow-up | |
| Splenic sequestration | 8 (19) |
| Cholecystitis | 1 (2.4) |
| Osteomyelitis | 8 (19) |
| Hemolytic crisis | 5 (11.9) |
| Aplastic crisis | 6 (14.3) |
| Thromboembolic events | 3 (7.1) |
| AVN | 1 (2.4) |
| VOC | 32 (76.2) |
| Sepsis | 1 (2.4) |
| Comorbidities | |
| G6PD deficiency | 9 (21.4) |
| Asthma | 11 (26.2) |
| CVS diseasesd | 2 (4.8) |
| Endocrine diseasese | 3 (7.1) |
| Developmental delay | 2 (4.8) |
| OSA | 1 (2.4) |
| Miscellaneousf | 4 (9.52) |
| Having≥1 comorbidities | 22 (52.4) |
| Medications before ACS episodes | |
| Hydroxyurea therapy at baseline | 1 (2.4) |
| Folic acid | 39 (92.9) |
| Penicillin V* | 28 (66.7) |
| Simple transfusion* | 2 (4.8) |
| Albuterol* | 5 (11.9) |
*Missing details were excluded, d HTN and CHD, e Type-1 DM, rickets, and SIADH, f Epilepsy, achalasia, autism, and eczema. ACS=Acute chest syndrome, SCD=Sickle cell disease, VOC=Vaso-occlusive crisis, AVN=Avascular necrosis, G6PD=Glucose-6-phosphate dehydrogenase, CVS=Cardiovascular system, OSA=Obstructive sleep apnea, IQR=Interquartile range, SD=Standard deviation, HTN=Hypertension, CHD=Coronary heart disease, DM=Diabetes mellitus, SIADH=Syndrome of inappropriate secretion of antidiuretic hormone