Clinical Correlates of Acute and Chronic Pulmonary Complications in Children with Sickle Cell Disease from Madhya Pradesh, India: A Cross-sectional Study

Abstract

Introduction: Sickle Cell Disease (SCD) is one of the most common hereditary haemoglobinopathies globally. Patients with SCD are at a high risk of pulmonary complications. However, there have been very few studies in literature regarding the spectrum of pulmonary involvement and its association with clinicotherapeutic parameters in Indian patients. Aim: To study the incidence of acute and chronic pulmonary complications in children with SCD and to estimate the association of various risk factors with pulmonary complications. Materials and Methods: The present observational crosssectional study was conducted in the Department of Paediatrics, Mahatma Gandhi Memorial Medical College (tertiary care hospital) and Maharaja Yeshwantrao (MY) Hospital, Indore, Madhya Pradesh, India, from June 2021 to July 2022. A total of 100 patients with SCD below the age of 18 years attending sickle-thalassaemia Outpatient Department (OPD) and Inpatient Department (IPD) with pulmonary symptoms admitted were enrolled in the study. Clinical history, physical examination and laboratory findings were noted as per the preconstructed proforma. Data were statistically analysed with unpaired t-test and Pearson’s Chi- square test. Results: A total of 100 patients with SCD were enrolled and the mean age of the study subjects was 9.27±3.39 years. Nearly equal distribution of males (n=52) and females (n=48) were seen with male to female ratio of 1:1.08. Incidence of complications was seen very high in homozygous sickle disease (63%) followed by only 40% in Haemoglobin S (HbS) beta thalassaemia. Most common complication noted in our patients was Acute Chest Syndrome (ACS) (57.7%), followed pneumonia (20%), sympneumonic effusion (15.6%) while only 6.7% had pulmonary hypertension. A statistically significant association (p-value<0.05) was found between compliance to Hydroxyurea therapy and frequency of Vaso-occlusive Crisis (VOC) episodes with pulmonary complications status. Conclusion: In the present study, most frequent acute pulmonary complication noted was ACS followed by pneumonia and sympneumonic effusion. Frequency of VOCs episodes was significantly associated with increased risk of developing pulmonary complications.