Lung Function Abnormalities in Sickle Cell Anaemia

Sickle Cell Disease and Sickle Cell Anaemia

Sickle cell haemoglobin (HbS) results from an autosomal recessively inherited mutation in which the 17th nucleotide of the beta globin gene is changed from thymine to adenine and the amino acid glutamic acid is replaced by valine at position 6 in the beta globin chain. [1, 2]Sickle cells have a reduced deformability and are easily destroyed, causing occlusion of the microcirculation and a chron...

Sickle cell anaemia.

PAEDIATRIC LUNG DISEASE Pulmonary function abnormalities in children with sickle cell disease

Background: Adults with sickle cell disease (SCD) have restrictive lung function abnormalities which are thought to result from repeated lung damage caused by episodes of pulmonary vaso-occlusion; such episodes start in childhood. A study was therefore undertaken to determine whether children with SCD have restrictive lung function abnormalities and whether the severity of such abnormalities in...

Effect of Lisinopril on Microalbuminuria in Sickle Cell Anaemia Children: A Single-Blind Randomized Controlled Trial

Background Sickle cell nephropathy is a major cause of morbidity and mortality in sickle cell anaemia (SCA). Proteinuria contributes to progression of renal damage. Icroalbuminuria is an early feature of SCN and progression to advanced kidney damage is delayed if regression is achieved with angiotensin converting enzyme inhibitors. We aimed to ...

Computed tomography and pulmonary function abnormalities in sickle cell disease.

The aim of this study was to determine whether patients with sickle cell disease (SCD) in steady state had pulmonary abnormalities seen on high-resolution computed tomography (HRCT) and whether any abnormalities correlated with contemporaneously diagnosed lung function abnormalities. A subsidiary question was whether the results of a noninvasive measure of haemolysis (end-tidal carbon monoxide ...