Clinical profiling of sickle cell disease patients with respect to pulmonary hypertension

Abstract

<p class="abstract"><strong>Background:</strong> <span lang="EN-IN">Sickle cell disease (SCD) is the commonest heritable hematologic abnormality affecting humans. Pulmonary hypertension is documented in patients of SCD by both non-invasive methods and catheterization, with incidence reported to be as high as 20%-63%. However, pulmonary hypertension has not been studied as complication of patients of sickle cell disease in India</span>.</p><p class="abstract"><strong>Methods:</strong> <span lang="EN-GB">A total of 94 (54 SS and 40 AS) more than 12 years old cases </span><span lang="EN-IN">diagnosed to have sickle cell disease by hemoglobin electrophoresis</span><span lang="EN-GB"> were recruited. All the participants were assessed with complete history, general and systemic examination, hematological and biochemical tests, X-ray chest, ECG, pulmonary function tests and 2D echocardiography and Doppler studies. </span><span lang="EN-IN">Cases were observed in 2 groups: SS cases and AS cases. </span><span lang="EN-GB">Sixty four</span><span lang="EN-IN"> age/sex matched healthy close relatives of the cases with ‘AA’ Hb electrophoresis pattern were the controls. </span><span lang="EN-IN"> </span></p><p class="abstract"><strong>Results:</strong> <span lang="EN-GB">Five SS cases were detected to have pulmonary hypertension. Pulmonary hypertension was not detected in any AS case or control. Pulmonary hypertension was more common in females (3/23, 13%) than males (2/26, 7.7%). </span><span lang="EN-IN">The mean age of the SS cases with PH (32±5.15) was found to be significantly higher than that of the SS cases without PH (24.2±6.21) (p<0.01)</span>.</p><p class="abstract"><strong>Conclusions:</strong> <span lang="EN-GB">The incidence of PH was low in this study, as compared to western studies. This may be since most of the cases of SCD being in remote areas having no access to health care facilities</span><span lang="EN-IN">. </span></p>