Table 2.
The prevalence of selected clinical consequences of SCD.
Clinical event | Prevalence | References |
---|---|---|
Haemolysis | ||
Anaemia | Chronic | [57–59] |
Cholelithiasis | Prevalence is 40% by adolescence | [60, 61] |
Aplastic anaemia | Associated with parvovirus B19 infection | [61–63] |
Hyperhemolysis | Limited reports from Africa | [64–67] |
| ||
Vasoocclusion | ||
Pain | More than 60% patients Most common cause of admission Frequent pain is a risk factor for mortality |
[22, 23, 68, 69] |
Acute splenic sequestration (ASS) | Frequently occurs before the age of 3 yrs | [23, 70, 71] |
Leg ulcers | Prevalence is 10–25% adults | [72, 73] |
Priapism | Prevalence is 10–40% males Occurs frequently in 5–14 years age group |
[74] |
| ||
Organ dysfunction | ||
Neurological events | ||
Stroke | Prevalence is 10% in children risk factor for mortality High rate of recurrence Leads to poor quality of life |
[75] |
Cognitive/silent | Prevalence is 20% Risk factor for overt stroke Leads to impairment of executive function |
[76–79] |
Retinopathy | Prevalence is >30% in HbSC | [80] |
Chest | ||
Acute chest syndrome (ACS) | Prevalence is 40% Occurs frequently in children Has severe consequences in adults 12.8 per 100-patient years 59 |
[54–56] |
Pulmonary hypertension | Prevalence is 30% Risk factor for mortality |
[79, 81–84] |
Avascular necrosis of femoral head | Prevalence is 10–50% in adults | [85–87] |
Renal disease | Prevalence of chronic renal failure is 5%–20% | [88] |
| ||
Infections | ||
Malaria | There is low prevalence of malaria in SCD. However, when malaria occurs in SCD it is associated with increased risk of morbidity due to severe anaemia and mortality | [89, 90] |
Bacterial infections | 10% children under 5 years | [91] |