TABLE 1.
Description of the Cameroonian SCD cohort.
| Variables | Median (95% CI) or frequency (%) | Min–max | P-value | Observation (n) |
| Age (years) | 15.5 (15–16.5) | 2–58 | 0.1123 | 413 |
| Gender | M/F: 210/203 | 413 | ||
| Clinical Events | ||||
| VOC (n/year) | 2 (2–3) | 0–80 | 0.4949 | 412 |
| Hospitalisation (n/year) | 1 (1–1) | 0–40 | 0.2676 | 405 |
| Transfusion | Y/N: 76.8/23.2 | 410 | ||
| Anthropometric | ||||
| Body mass index (kg/m2) | 17.6 (17.2–17.9) | 10.6–32 | 0.1155 | 413 |
| Systolic blood pressure (mmHg) | 103 (101.5–104) | 72–156 | 0.8637 | 409 |
| Diastolic blood pressure (mmHg) | 58 (57–59) | 37–93 | 0.0162 | 409 |
| Haematological | ||||
| Hb (g/dl) | 7.55 (7.4–7.7) | 3.5–13.1 | 0.4905 | 406 |
| MCV (fl) | 91 (90–92) | 61–125 | 0.9905 | 406 |
| Platelets (109/l) | 368.5 (355–382) | 29–1,078 | 0.8483 | 406 |
| Leucocytes (109/l) | 12.65 (12.2–13.2) | 4–49.8 | 0.0471 | 406 |
| Lymphocytes (109/l) | 5.2 (4.95–5.4) | 1.4–22.1 | 0.0219 | 406 |
| Monocytes (109/l) | 1.5 (1.45—-1.6) | 0.1–8.1 | 0.0009 | 406 |
| Granulocytes (109/l) | 4.5 (4.25–4.75) | 0.2–24.3 | 0.0976 | 406 |
| HbA2 (%) | 3.15 (2.9–3.3) | 0–18.2 | 0.0008 | 412 |
| HbF (%) | 9.95 (9.3–10.65) | 0–37.2 | 0.2026 | 412 |
| Alpha-thalassemia Genotypes (%) | <0.0001 | 339 | ||
| αα/αα | 57 | 194/339* | ||
| αα/α3.7 | 32 | 109/339* | ||
| α3.7/α3.7 | 11 | 36/339* | ||
| HBB Haplotype (%) | <0.0001 | 352 | ||
| Ben/Ben | 55 | 195/352* | ||
| Ben/Cam | 26 | 92/352* | ||
| Ben/Atypical | 7 | 25/352* | ||
| Cam/Cam | 7 | 23/352* | ||
| Cam/Atypical | 2 | 7/352* | ||
| Atypical | 1 | 3/352* | ||
| Other haplotypes | 2 | 7/352* | ||
| Renal functions | ||||
| Serum creatinine (mg/l) | 6.8 (6.5–7) | 2–13.8 | 0.3958 | 404 |
| Crude-albuminuria (mg/dl) | 51.5 (47–55.5) | 3–1,180 | <0.0001 | 407 |
| Normal (%) | 37 | 149/407 | ||
| Micro-albuminuria (mg/dl) (%) | 61 | 248/407 | ||
| Proteinuria (mg/dl) (%) | 2 | 10/407 | ||
| eGFR (ml/min/1.73 m2) | 155.4 (151.6–159.2) | 58.9–290.7 | 0.5715 | 404 |
| Normal (%) | 23 | 93/404 | ||
| Glomerular hyperfiltration (%) | 71 | 287/404 | ||
| Kidney failure (%) | 6 | 24/404 |
*Number of individuals, not alleles. VOC, vaso-occlusive crises; Hb, haemoglobin; MCV, mean corpuscular volume; HbA2, Haemoglobin A2; eGFR, estimated glomerular filtration rate. In all cases, Wilcoxon tests were performed to check whether the parameter falls within the confidence interval (CI) for p-value greater than 0.05, except for alpha-thalassemia genotypes and the HBB Haplotype, for which adjusted (χ2 p-values were computed to check whether these proportions follow a uniform distribution for p-value greater than 0.05. In the second column, median values are followed by CI, whereas proportions are not.