Sickle Lung Disease Long-Term Consequences and Prevention.

Abstract

Sickle cell disease (SCD) is of global significance due to its severity and occurrence worldwide. Inheritance of the abnormal hemoglobin structure contributes to microvascular events that underlie the development of the multi-systemic complications seen in the disease pathogenesis. Pulmonary complications are common and heterogeneous including pulmonary hypertension, sleep-disordered breathing and lung function abnormalities. Lung function abnormalities commonly reported among children with SCD living in Africa are restrictive impairments. However, in high-income countries, reports suggest that obstructive lung function impairment is more predominant. The exact process that contributes to lung disease in SCD must be continuously explored and large-scale longitudinal studies employing multiple lung function methods are needed urgently. Lung disease-modifying agents need to be explored to help slow down or prevent the occurrence of pulmonary function abnormalities.