Review Article

Sickle Cell Disease: New Opportunities and Challenges in Africa

Table 2

The prevalence of selected clinical consequences of SCD.

Clinical eventPrevalenceReferences

Haemolysis
 AnaemiaChronic[5759]
 CholelithiasisPrevalence is 40% by adolescence[60, 61]
 Aplastic anaemiaAssociated with parvovirus B19 infection[6163]
 HyperhemolysisLimited reports from Africa[6467]

Vasoocclusion
 PainMore 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 ulcersPrevalence is 10–25% adults[72, 73]
 PriapismPrevalence is 10–40% males
Occurs frequently in 5–14 years age group
[74]

Organ dysfunction
 Neurological events
  StrokePrevalence is 10% in children risk factor for mortality
High rate of recurrence
Leads to poor quality of life
[75]
  Cognitive/silentPrevalence is 20%
Risk factor for overt stroke
Leads to impairment of executive function
[7679]
  RetinopathyPrevalence 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
[5456]
  Pulmonary hypertensionPrevalence is 30%
Risk factor for mortality
[79, 8184]
  Avascular necrosis of femoral headPrevalence is 10–50% in adults[8587]
  Renal diseasePrevalence of chronic renal failure is 5%–20%[88]

Infections
 MalariaThere 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 infections10% children under 5 years[91]

Modified from [92, 93].