As new therapies emerge, potentially leading to disease amelioration or cure, it is of paramount importance that the significant burden of SCD in resource-poor countries is properly recognised.
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In the Stroke Prevention in Sickle Cell Anaemia (STOP) trial, the risk of stroke among children with high TCD velocities was reduced by 90% by maintaining Hb S concentrations at Severe abdominal pain, often unresponsive to analgesia and associated with intestinal ileus and acute ischaemic colitis, is termed ‘girdle syndrome’ owing to the circumferential distribution of pain.
A high index of suspicion and early implementation of supportive therapy (including emergency red cell exchange transfusion, analgesia and fluids) may prevent irreversible ischaemic damage to the gut.65 Priapism, defined as prolonged penile erection lasting 4 h, is a urological emergency and can result in fibrosis of the corpora cavernosa and permanent erectile dysfunction if not treated early.
The last are characterised by pain and a systemic inflammatory response that may be severe, episodic and unpredictable.
Some of the more common acute clinical and laboratory features of SCD are summarised in table 1, along with descriptions of current approaches to their management.
DNA-based methods are commonly used to confirm the diagnosis of SCD in more complicated cases.28 Since SCD was first described a century ago, a great deal has been learnt about its pathophysiological consequences.
Under conditions of hypoxia, acidity and cellular dehydration, the polymerisation of Hb S within erythrocytes leads to their deformation into the characteristic ‘sickle’ shape.
While the treatments of common complications are summarised in table 1, pain relief, hydration, aggressive treatment of sepsis and blood transfusions remain central to acute management.
Hydroxycarbamide (or hydroxyurea) remains the only agent that has been proven to reduce the number of episodes of painful crises, ACS and hospitalisations in randomised control trials in adults,71 school-age children72 and infants73 with SCD.