Potential Future Therapeutic Options

The lung is the largest epithelial surfaee in the human body, the alveolar surface area being estimated at 70 m. It is highly complex and constitutes a three dimensional structure of unique cells lining a series of bifurcating 

Candidate genes with significant SNP associations Potential role in acute lung injury 

Atplal Maintains electrochemical gradient of Na /K ions across plasma membranes essential for osmoregulation role in clearance of pulmonary oedema 

Alox5 Encodes arachidonate-5-lipoxygenase enzyme, which converts arachidonic acid to leukotrienes— important mediators of a number of inflammatory and allergic conditions 

Hrhl Gene encoding histamine receptor HI. Histamine mediates contraction of smooth muscle and an increase in capillary permeability due to contraction of terminal venules 

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Both vitamin E supplements and splenectomy have been suggested as potential therapeutic options, but neither has proven to be beneficial. Gene therapy is a potential future treatment under development but would only be clinically justified in the most severe cases affected by chronic hemolysis. 

An added benefit is that inhalational therapy reduces potential undesirable negative side effects of treatment when compared to systemic delivery options. Singleagent tolerability via inhalation has been demonstrated in asthmatic and idiopathic pulmonary arterial hypertension (IPAH) patients. The current and future program is designed to test and confirm general efficacy as single therapeutic inhaled agent. 

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